TRACHEOESOPHAGEAL FISTULA

DIAGNOSIS AND DESCRIPTION

Our initial NICU consultation offered us little hope. And the somber, blank expression on the nurse’s face while delivering our news, has been burned into my memory. With her words, our son’s birthday became the best and worst day of my life all in one.

Leaving the NICU, I didn’t think our son would survive, and I couldn’t wrap my mind around it. How could a baby who looked so perfect and healthy be so broken on the inside?

I was empty and incomplete. I just wanted to hold him, and our separation paired with the distance to the maternity ward, was unbearable.

When we finally reached my room, we were greeted by an unfamiliar nurse expecting two people in a celebratory mood. Smiling ear to ear she greeted us, “Hi! Congratulations! How are you feeling, Momma?” “Not good,” was all that I could get out.

I had been living in the hospital for 6 weeks, but in another ward. Here, strange nurses were asking me questions about myself that I wished they already knew. I wondered why, after all this time (and in a time like this) am I being asked questions about myself?

The new and unknown nurses teamed up to transfer me from my OR bed to my new bed. I can’t lie, it really freaking hurt. All feeling had returned to my post-op body. Everything was setting in. And just then, nurse Peggy walked in. I burst into tears.

Nurse Peggy was one of my antepartum nurses and more. In addition to providing care for the previous six weeks, she had become a sort of maternal nurturer to me. And to no surprise, here she was, hugging me like a daughter, while letting me sob into her shoulder.

Patient with me while I tried to stop crying, she was still in the room when the surgeon arrived. I told her she didn’t have to stay. Of course, she didn’t listen.

Now, it is no exaggeration to say our whole life changed when the surgeon began speaking.

Smiling and confident, he said the words we were least expecting. Our son’s condition was treatable with surgery. And more, he assured us our son’s life-saving operation had a near 100% survival rate.

Looking at each other in disbelief, my husband and I tried to make sense of his words. The surgeon continued, “Surgery needs to happen soon. These babies can only get nutrition through an IV and it doesn’t keep them satisfied for long.” Our son’s operation was scheduled for the following morning.

We were warned our son would be on a respirator following surgery, and would need additional exams to determine whether or not he had further, associated complications.

In turn, we asked him questions about our son’s future that he couldn’t answer with certainty. But, at last, we had reason to believe that our baby could survive. And to top it off, we were given permission to return to the NICU. We were finally going to be with our son.

It took months, maybe years, for the fear and shock of our son’s diagnosis to wear off of my husband and I. We’ll be grateful everyday for the rest of our lives, but we still don’t understand why we were given zero hope that morning in the NICU.

TE fistula with esophageal atresia image curtesy of Akron Children’s Hospital

So what is tracheoesophageal fistula? Here are some quick facts, with links to the supporting medical websites below:

Tracheoesophageal fistula is a birth defect where the esophagus and trachea (airway) have an abnormal connection
TE fistula often occurs with esophageal atresia. Meaning the esophagus grew in two parts. One part connected to the mouth, the other part connected to the stomach (see below image)
The only way for these babies to eat and drink on their own is with corrective surgery. The surgery needs to create a full and secure connection between the mouth and stomach via the esophagus, and between the airway and lungs via the trachea
TE fistula occurs in approximately 1 in 5,000 babies
Up to one half of babies with TE fistula also have another serious birth defect including heart, digestive tract, kidney, and muscular/skeletal problems. X-rays and further testing would be necessary
TEF was a “hopeless congenital anomaly” not long ago. The first successful repair surgery was not until 1941. With survival rates nearing 100% only in the last few decades

For TEF SERIES PART 1: PREGNANCY AND DISCOVERY, visit:

https://believableshe.com/2021/06/03/series-tracheoesophageal-fistula-from-nicu-to-two/

REFERENCES

https://www.stanfordchildrens.org/en/topic/default?id=tracheoesophageal-fistula-and-esophageal-atresia-90-P02018

https://www.akronchildrens.org/kidshealth/en/parents/te-fistula-ea-html.html

https://www.medscape.com/answers/186735-99657/when-was-the-first-successful-repair-of-tracheoesophageal-fistula-tef

PREGNANCY AND DISCOVERY

***Please be advised. This story contains medical descriptions.

When our son was born with a pair congenital defects called tracheoesophageal fistula (simply known as TE Fistula or TEF) and esophageal atresia, we had no warning. My husband and I found out the day he was born, and we were offered little more than surgical plans.

Desperate to know what this diagnosis meant for our son’s future, we scoured the internet for real-life stories. What we found couldn’t even be considered a hand full, but they were our life-line.

With hopes of providing peace to families who are, or will, find themselves in a similar situation, I am sharing our story through a series of posts called, Tracheoesophageal Fistula – From NICU to Two

If you, your child, or loved one has gone through this diagnosis, I encourage you to comment, ask questions, and share your experience. Your stories matter.

My pregnancy was riddled with complications. I begin our story here because many of these complications disguised themselves as common conditions that do not indicate TEF. Looking back, it is now clear that I had a very specific combination of factors resulting from our son’s defects.

In my second trimester, I was diagnosed with a case of placenta previa. Initially, the condition was described as mild enough to correct itself. So when I began hemorrhaging at twenty-six weeks, it surprised me and my OB.

Needing constant observation, I was prescribed hospitalized bedrest. While hospitalized, I had numerous ultrasounds. None of which detected TEF.

As my pregnancy progressed, I also began leaking amniotic fluid. Although concerning to my doctor and nurses, I had an unusually large volume of fluid, so they assured me our baby was safe.

The danger of too much fluid, however, lies in the fact that it can cause the uterus to expand too fast. This expansion caused my placenta to begin detaching from my uterine wall. This detachment caused my bleeding to become more frequent. And my frequent bleeding led to an emergency C-section at 34 weeks.

In utero, babies practice swallowing by “drinking” amniotic fluid. TEF meant that our son’s esophagus did not connect his mouth to his stomach, so he could not practice this developmental step. This lack of practicing caused fluid to build up, and led to severe bleeding. This is the warning that was missed.

I was anesthetized for my emergency C-section. Due to risk of severe bleeding, I could not have a spinal block and my epidural made me sick. Not being numbed enough, I felt the first cuts into my abdomen, and I begged to be put under.

The first thing I remember after waking, was a nurse telling me I had a beautiful baby boy with sandy blonde hair. She was kind and smiling, and assured me that I could see my baby soon.

Though feeling hazy, it didn’t seem too long before my husband was allowed to enter post-op. Joined by a nurse (or two) we were brought to our son. Still laying in my hospital bed, I was wheeled through the halls to the NICU. I remember feeling disappointed that I wasn’t awake to greet my son, but so excited to hold him for the first time.

Being wheeled in on a bed drew attention, and I was uncomfortable feeling every eye on me as we entered the NICU. But then I saw him. Lying on his tummy, in his incubator, beautiful as could be.

Because of my complicated pregnancy, we knew he would be premature. We were previously prepped by NICU nurses, and his IV, mass of cords, and beeping monitors didn’t scare us. But I still remember shaking while reaching through the incubator hole to touch his sweet baby back as gently as possible. I remember telling him that he was perfect.

There was a full team of nurses surrounding us and our baby. A few in scrubs, and one in office clothes. Given the circumstances, the amount of people didn’t seem strange. But then the woman in office clothes started talking.

I am paraphrasing today, because the next several minutes were honestly a blur. But she said something to the effect of, “We need to talk. There has been an unforeseen complication.”

Her face was serious. Her words were frightening. Still recovering from anesthesia, her words came at me as fractured bits and pieces.

“You can’t hold him right now.”

“We couldn’t get a feeding tube down his throat.”

“He needs more tests.”

“Tracheo…fistu…”

“He’ll need an operation.”

“We don’t know… We don’t know… We don’t know…”

“Time to go back to your room. The surgeon will be in to see you.”

With only moments to spend together, I told my son I loved him, and he was still perfect. Then, confused, heartbroken, and expecting the worst, I was wheeled back to the maternity ward without him.

For TEF SERIES PART 2: DIAGNOSIS AND DESCRIPTION, visit:

https://believableshe.com/2021/06/08/series-part-2-tracheoesophageal-fistula-from-nicu-to-two/