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Leigh Ann

From Leigh Ann’s Mom:

We found out about Leigh Ann’s heart condition at her 20 week ultrasound. The doctor saw something was missing from her heart and told me not to worry, and that doctors could fix her. We were sent to Texas Children’s Hospital three days later for a fetal echo. That is where we found out she had HLHS (Hypoplastic Left Heart Syndrome). It was so foreign to me. I knew nothing about heart conditions, let alone HLHS. We spent the next few months mentally preparing ourselves, researching, attending multiple doctor appointments, and decorating her nursery hoping she would get to actually come home to it one day.

She was born a week early by c section because she was breach and went immediately to the CVICU (Cardiovascular Intensive Care Unit) at birth. On day seven, Dr. Fraser did her Norwood surgery. One week later she coded and we thought we lost her. She remained intubated for the next two weeks while doctors tried to figure out what happened. It is still unknown. She never got to the leave between surgeries because she continued to get NEC, which is basically when the intestines start to die due to lack of oxygen. So she lived on TPN until the Glenn surgery was done four months later on July 11th. Tell a mom you can’t feed her baby? That was the hardest thing for me.

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We remained in the ICU for two more months after because of her lung pressures and because she needed to be on Nitric oxide for several days. We also continued to battle reflux issues, so she received her GJ tube, which was a blessing in disguise. Her stomach just was not ready to be fed yet. A week before we discharged she had a cath done to coil collateral vessels that were stealing oxygen from her heart. Her oxygen saturation was immediately better and she was no longer a purple color; she was pink!

Edited Leigh Ann in hosptial bed

They discharged us from the ICU instead of the step down unit because the nurses knew Leigh Ann well and felt it was best they discharged her themselves. September 29th we got to go home! She has been home every day since, besides a 24 hour hospital stay over Christmas for the flu. She is almost 100 % fed by mouth now, but still has her tube for night feeds. She crawls, talks, and is happy as can be. She receives in-home nursing twenty-six hours a week and adores her nurse. She receives speech, physical, and occupational therapy twice a week as well. She came home on thirteen medications and now is down to four meds. It makes such a difference.

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In September she will be having an elective cath to balloon open her arch and possibly coil more collateral vessels. They will also check her lung pressures as we near the Fontan procedure in a few years.

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How Are Things Now?

Addie

I never really know how to explain Addie’s diagnosis to people. Just like Eloise, it’s complicated, random, and rare. Addie has left atrial isomerism heterotaxy. We first heard the words “your baby will need open heart surgery” when I was about 16 weeks pregnant. Doctors said, “One surgery and she will be done”, and “Less than a 1% chance it’s anything else.” While painting Addie’s nursery dresser and imagining each drawer filled with sweet outfits for our little girl, I got the call. It was one that made me lose my breath and feel faint. Our baby girl had heterotaxy and there were many defects in her heart. Josh, my husband, came home from work and we talked about heterotaxy and wrestled to understand that our baby would be enduring a life-long struggle. It wasn’t until after Addie was born that we knew the full extent of her defects which include: single left-sided SVC, interrupted IVC with hemiazygos continuation, separate hepatic veins, unbalanced atrioventricular septal defect, left ventricular outflow tract obstruction, hypoplastic aortic arch/ coarctation, and severe atrioventricular valve regurgitation.

The cardiologist had prepared us for the terrible possibility of having only a couple days with our sweet girl. As I struggled to understand the implications of these terms, I set goals in my mind for a few things I hoped to do with her if we only had a short time together. The main thing on my mind was to sing her “Happy Birthday”, so in the delivery room with our friends and family around we sang “Happy Birthday”.  Another was to brush her hair and luckily she was born with lots! I cherish that little comb 15 months after I first used it. It was the mother-daughter moment I had been hoping for. We have been so lucky with all the time and wonderful moments shared with Addie. Two open-heart surgeries and several procedures later, our daughter is an active, bright, happy toddler.

Eloise

Eloise was prenatally diagnosed at 18 weeks with heterotaxy, and more specifically, left atrial isomerism heterotaxy. “Hetero” means different and “taxy” means arrangement. Her stomach is on the opposite side and she has EIGHT spleens, among other organ abnormalities. Along with her different arrangement of organs, the most serious of all her complications are congenital heart defects associated with heterotaxy. In just her first year of life she has undergone countless procedures and two open heart surgeries — one to place a pacemaker when she was only two weeks old, and another to complete her full repair when she was nine months. My baby is a FIGHTER and I am constantly amazed at her feisty spirit through it all.

More Specifics About Her Heart

Eloise’s heart rate was 62 bpm for most of my pregnancy (a heart healthy baby should be around 140 – 160 bpm) and doctors didn’t know if she would even make it. She is missing an electrical node which the top and bottom chambers of the heart use to communicate (a defect called complete heart block). Because of this, she had a dual chamber pacemaker placed when she was only two weeks old. She was born with three very large holes in her heart. One was an atrial septal defect (ASD), which is a defect in the septum of the top two chambers of the heart. The other two were ventricular septal defects (VSDs), defects in the septum of the bottom two chambers. In her case the holes were so big that it was almost as if there was no septum there at all. This means that the oxygenated and non-oxygenated blood going to and from the lungs was mixing. This made her heart work extremely hard and she had a ton of trouble breathing. In fact, her heart worked so hard that at the time of her full repair at nine months old, it was 50% bigger than it should have been. Other issues she had that I’ll generalize for now: PAPVR, a thickened pulmonary valve, tricuspid valve regurgitation, pulmonary stenosis, interrupted IVC with azygos continuation, malrotated intestines, and a duplication cyst on her stomach.

Eloise truly is our little miracle baby and is a thriving one year old today. She is so loved.