Aunt Becky’s daughter, Amelia, is the sole reason that The Band exists. Amelia, like so many of our children, was born with a birth defect called an encephalocele. She’s gone on to vigorously beat neurosurgery and the odds stacked firmly against her.
So it’s time to Band Back Together for Birth Defects. Share your stories. Tell your tales. We need to learn about the birth defects that have touched YOUR life. Let’s rock out and tell the world OUR stories.
It’s YOUR turn, The Band!
She was born in September. It was hot. And in 12 weeks, we would be speaking a new language.
My baby was just 2 days old when we learned that our “perfect” world was not to be. My whole life I dreamed of motherhood. It’s what I wanted to do, more than anything. Be a good mom. Raise a family. Teach a little person about my faith.
Rewind to my college days – I was one of those students that changed majors more than once. First I was going to be a teacher; I majored in elementary education. Then after two summers directing a summer camp, I learned that wasn’t really for me. I wasn’t a fan of the parental drama.
Then I was an accounting major. I was going to be a CPA. Then I realized I needed more and ended up an accounting minor with a business administration major in both finance and marketing.
But none of that told me what I wanted to do with my life.
All I cared about was that someday I was going to get to be a mom.
I married my high school sweetheart. We’ve been together since 1992. We got married. Got jobs and got ready to have the “perfectly perfect normal life” that we’d always planned. God has a funny sense of humor sometimes.
And then almost 3 years after we got married, 11 years after we first met, we had Natalie.
And then in 2 days, our world was rocked.
Her bilirubin level of 22 just would not go down.
Our family doctor made the decision to send her to a bigger hospital in a bigger city. He told us that his ego was small enough that he knew that she could get better care and more answers there. The ambulance drove away, and we felt terribly alone. She was whisked away to a NICU and our baptism into a world of medicine was begun.
Our baptism was truly begun with a Baptism.
Natalie was taken to a NICU in a town an hour away from where she was born.
My baby sister Bridget was taken from the same hospital to an ICU just 21 years before, after she was born. She never returned home.
I know that was on my dad’s mind.
My baby sister was born with Transposition of the Great Vessels. She lived 7 days. She was operated on by a fantastic surgeon, who just happened to be the very same surgeon who would perform our daughter Natalie’s very first surgery, a cholangiogram.
The surgeon did his fellowship at Children’s Memorial in Chicago. This same place where Natalie would someday have her liver transplant.
So many coincidences…
Our NICU surgeon made the comment to us that is the title of this entry, “We think it’s Biliary Atresia, but that’s really bad so hopefully that’s not it…”
I don’t hold a single ounce of ill will toward the man. Natalie’s case confused everyone.
She was born with a gallbladder. Albeit a shriveled, ugly, non-working gallbladder. But a gallbladder, nonetheless. And that’s just not common in Biliary Atresia.
In “classic” biliary atresia, by the time most kids are born, their bile duct structure (gallbladder included) has shriveled up and is not working. But Natalie was born 5 weeks early, and it’s a progressive disease – meaning it gets worse as time goes by.
Back to my dad. He’d seen things end badly for his child. I know he had his grandbaby’s soul in mind when he told me that we needed to baptize her.
I am a Catholic.
New babies = Baptism is second nature for me. But the reality of this was too much to bear. I’d had the story of Bridget’s birth and death memorized. I was 4 when she died. Her death is my very first memory. Her death prepared me for my future role, of that I am now certain. But in that moment, I could not face it.
Here I was 2 days after the birth of my child.
I’d had pre-eclampsia. I was induced just two days before following 35 weeks of pregnancy. I was a swollen, puffy blob, having gained 30 pounds in the last month of my pregnancy alone. My husband said that the moment I gave birth to our daughter, my blood pressure had skyrocketed to 250/204. No joke. I was given magnesium to prevent a stroke or a seizure.
My mind was so fuzzy.
I was still in shock.
I wanted my “normal” life back!
I was in denial.
This wasn’t happening.
Why was my dad suggesting that we baptize my baby? Did he think she was going to die? I dug my heels in. (At least I tried to. I could only fit my fat feet into a chewed up pair of black sandals – that my German Shepherd had gotten ahold of.)
So my dad did the good dad thing. He did the responsible thing. He overruled me.
He called our dear friend, the priest. He had been the priest at our church when Jason and I met (we met at church, have I mentioned that?). In walked my dad and the priest, through the sliding NICU doors and over to Natalie’s bassinet. I watched it in slow motion. I remember it in slow motion. I don’t even think I have any pictures of the moment that is forever etched in my mind.
At the time of Natalie’s first surgeries, we had not created a website for our kid, Facebook was not around, and MySpace was a name I called my bedroom. What I am saying is that I did not document my thoughts and feelings at that time, the way that I do now. Not that you would have wanted me to. You see, for the first 3 weeks of Natalie’s life, we lived in our home and visited our baby in the NICU.
I cried every time I left her, which was every evening and every shift change. I’d start crying in the elevator leaving the NICU and finish up half an hour later as we pulled into our driveway.
We did not know in the beginning that Biliary Atresia would be Natalie’s final diagnosis. She began her stay in the NICU under the UV lights, like any other jaundice case. We’d work on “normal” things, like trying to get her to eat. The first day it was 15 ml every 3 hours, then 20ml, then we finally worked up to a whopping 30 ml. I was encouraged, cheered on even, to keep breastfeeding. And so I did. It gave me a purpose. A sense of control.
And when you can’t control anything, you’ll control that one thing with everything you’ve got.
I took to it like a champ. Strike that. I took to pumping like a champion dairy cow. I focused on finding ways to increase my milk production levels. I found special teas marketed themselves as “Mother’s Milk” tea. My loving husband was my biggest advocate. We’d walk down a long hall, into some section of the hospital that was no longer in use, except for the rare nursing mother pumping session. There was a room with 3 old chairs, a sink, paper towels and soap (for keeping supplies clean) and outlet. Not much more, except for some posters donated by a La Leche League USA. I’d plug my Medela Pump into the wall, take a seat on the old metal chair that was missing chunks of vinyl on the seat, and watch the milk rise in the bottles, feeling victorious as the ounces would climb higher and higher, knowing this was for my girl. The aches I felt, sitting hunched over, were worth it. This was for Natalie. This was making her stronger, this I could do.
After a few days, they did a full blood work-up. Her GGT level was around 1700 (normal is 5 – 55), letting the doctors know that something else was wrong.
They’d take her for an ultrasound. “Inconclusive.” She was born with a gallbladder, after all, and was stumping them.
After this happened 3 times, she had a HIDA scan, and then an open cholangiogram and also a biopsy, all on the day that she turned 2 weeks old.
That’s when our surgeon met with us and drew on a paper towel what we were looking at.
He had opened Natalie up, injected dye, and then tilted the table to watch the flow of the dye. And that’s when he said that he hoped that this wasn’t Biliary Atresia. He and the Pediatric GI attending to us in the NICU, both referred us to a wonderful team of doctors at Children’s Memorial in Chicago. And in the meantime, the biopsy slides were sent to Mayo clinic.
Mayo’s answer came back “Biliary Atresia.” But all other local doctors disagreed. So we got the slides back and took them with us to Children’s Memorial.
When we arrived in Chicago, it was like no place we’d ever been. There were bright colors everywhere. There were multiple waiting rooms with lively fish swimming in tanks. We were escorted to an examination room on the first floor. We thought that we must have been in the wrong place. There were no plaques or diplomas oh the wall. We met two doctors and told them we’d hear the term “breastfeeding jaundice.” The awesome doc, the head of the pediatric gastroenterology at the hospital, gave me a look like I’d just crawled out of a cave. “That is a myth. We’ve disproved it.” OK, I thought. Not going down THAT road with him. I had just had my first lecture by a genius, and I wasn’t a fan of lectures. But it let me know that we were in the right place. They knew their stuff. And above all else, they forbid me to feel guilty.
The fellow (also a doctor, so many levels of hierarchy at the hospital) took the slides and reviewed them. He asked the genius doctor to review them. Their first review said that she may have Cystic Fibrosis. They said it just did not present like “typical biliary atresia.” And so, sweat tests were done = “negative” was the answer. Genetic tests were sent away, and those took 6 weeks to get back. 6 long weeks of desperate waiting. The results came back negative.
After we had left the NICU, we waited for the other shoe to drop. We were still waiting. We waited for the bad news that we knew would come. Every inconclusive answer left me feeling more and more frantic. Genius doctor had told us that for a Kasai Procedure, the procedure to treat Biliary Atresia, to be most successful it must be done by the time the child is 12 weeks old.
The clock kept ticking.
Just a few days later we returned to Children’s and during a follow-up exam, Natalie happened to have a dirty diaper. Genius doctor took one look at it and re-diagnosed her with Biliary Atresia.
Who knew the answer was in the poop?
She was admitted and had another biopsy; it was again inconclusive. The kid is consistent. She then had another cholangiogram, this time a percutaneous type, which was then followed by a Kasai Operation on December 19 of that year. She was 11 weeks old. We’d gotten her surgery in by the time she turned 12 weeks.
Her new surgeon (also a genius), called hers a case of “correctable Biliary Atresia.” We’d learn later that things are not always what they seem and rarely are they as simple sounding as something called “correctable.” But, for the moment, we had an answer.
She got to come home on Christmas Eve.
Little would we know that within a year she would be listed for a liver transplant. I could not have guessed at this point that when she turned 17 months old that I would be giving part of my life to her.
But that’s a story for another day.