Sophia, 8 (Anchorage, AK) | Congenital Heart Defects

SOPHIA, 8 (Anchorage, AK) | VSD, Aortic Stenosis, Interrupted Aortic Arch

written by her mother, Talia

photographed by Laura Stennett Photography 

March 6,2008. That's the day everything changed.

My husband and I had gone to my 20-week appointment a couple of weeks before, and learned we were having another girl. Our older daughter Gabi was so excited, but my doctor was concerned about the baby's heart.

“It probably wasn't a big deal,” they said. It was hard to tell because she was laying on her right side, and wouldn't turn over so they could tell how the blood was flowing in her heart.

Fast forward to March 6, when the doctor came in to look at my ultrasound. As the tech listed off parts of the heart, all she said, over and over was "abnormal." I layed there and cried and cried and cried. My baby had a broken heart and I didn't know what that would mean for her.

I called Gabi's pediatrician and explained the situation to him, and was referred to our amazing cardiologist, Dr. Scott Wellman. Dr. Wellman was what I needed. He was straight to the point. I was not having this baby in Alaska, and she would have open-heart surgery shortly after her birth. 

Sophia was diagnosed with a large VSD (ventricular septal defect), aortic stenosis and interrupted aortic arch.

A ventricular septal defect is a hole in the wall separating the two lower chambers of the heart. Aortic stenosis is a narrowing of the aortic valve opening. It restricts the blood flow from the left ventricle to the aorta and may also affect the pressure in the left atrium.

An interrupted aortic arch means a small section of the aorta is missing. Children with interrupted aortic arch cannot pump blood normally to the lower body.

The thing about heart babies in Alaska is that there are no surgeons here, so you have to fly your family out (if you can afford it, most insurances only pay for the child and one adult escort) and stay in hotels/hospitals until it's time to come home.

Anyway, the plan was made for me to be induced 2 weeks before my due date, where Sophia would then be transferred from UW Medical Center where I would have her to Seattle Children's Hospital, shortly after her birth. Well, Sophia didn't like that idea, two weeks before I was supposed to fly to Seattle, I went in to pre-term labor. We immediately went to Seattle, because this baby was coming, but really she wasn't. Jason, Gabi and I hung out in Seattle for two weeks until July 7, 2008, we made our way to UW for our sweet girl to be born.

 I can't continue my story without talking about the wonderful people that have dropped everything to come to Seattle and help Jason, me and more importantly Gabi, not once, but twice and three times do some. My mom, my grandma, my sister, my aunt Linda and uncle Mark and my aunt Tina. I will forever be indebted to the love and support you all have given us over these 8 years.

After having Sophia (on July 8, mind you) she was taken to Seattle Children's Hospital. I was discharged the same day I had her, and we spent a week with her in the NICU.

On July 15, 2008, Sophia had her first open heart surgery. They were able to repair her heart, but she still had a bit of a heart murmur. She was kept open for a few days because her heart rate was funky, so we couldn't extubate her or hold her. I would rub her legs or her forehead. I would talk to her…I spent literally every waking moment with her.

Then came teaching her to eat. She was fed via a nasogastric tube because she could not be breast or bottle fed until after her surgery. This meant I was pumping breastmilk for her, a lot. I was hooked up to my breast pump every two hours. There was so much milk I filled a 60-lb cooler for the flight home. We were discharged on July 31 and headed home August 1. I was relieved to be home. There were medications to give and lovinox shots to give for a blood clot that formed, but we were taking our baby home.

It was a tough few months. Sophia was colicky, and I called my mom crying all the time. It was so hard. Then came November 2008. Routine cardiology appointment we thought, but it wasn't. Sophia and I were medivaced to Seattle because her aortic arch had severe blockage and needed a stent put in. We've had a lot of surgeries for Sophia, but this one was the scariest. This is when I first realized that my baby could be really sick.

Sophia went back to Seattle at 18 months for a balloon angioplasty in that arch and was put on blood pressure medication after that appointment. (Sophia just turned 8 and is still taking blood pressure medication twice a day).

 For the next three years, life was normal…or as normal as life can be with a child with a heart defect. I still cried a lot, I still worried a lot, but life was good. Sophia grew in to her feisty, stubborn self and we continued to see Dr. Wellman every 4 months.

In November 2012 (I'm good with dates, I kinda hate it), Sophia went for her check up before we went to Disney World. We were told Sophia would need another surgery, another angioplasty and at some point in the next 6-9 months, she would need another open-heart surgery.

I was crushed. Seriously, another open-heart?! How can this be? But I marched on. I wasn't going to show my girls my fear. In January 2013, Sophia and I flew to Seattle for a quick angioplasty and in February 2013, Sophia had her second open-heart surgery.

I don't do well with them, at all. I don't eat, I get grumpy and I just want to take all of this away from her. I would take this all on myself in a heartbeat if I could. 2013 was also the year Sophia had her Make-A-Wish trip. It was an amazing time, and a break from the reality of Sophia's condition.

Fast forward to August 2016. Again, we're told that within the next year, our Sophia will have another open-heart surgery. I'm sad, I'm mad, but most of all I'm numb. Sophia understands this now. A couple months ago, as we were driving in Seattle, I came to the sad realization that the next time we would return to Seattle would be for another surgery. I think Sophia did too. She laid her head on my shoulder and asked me what it would be like. She asked how long the surgery would be, how long would we be in Seattle. She asked how long would she be in the hospital. Instead of explaining it to a 4-year-old, on their level, I was explaining it to an 8-year-old and she got it. And that breaks my heart.

While I could end this with how I feel, which is angry, terrified and so sad, I'm going to end this with a look into our Sophia. Anyone that knows Jason and I know that we don't define Sophia by her heart defects. We have only told her she couldn't do something once; she can't play soccer with how physical it is becoming. But other than that, the sky's the limit.

Sophia is a ball of sunshine and energy. She is the happiest and most stubborn person I have ever met. She is strong and compassionate and she literally has the biggest heart of anyone I know. You would never guess she has had five heart surgeries if you met her. I honestly don't know what the future holds for. I don’t know how many more surgeries she will have, but I know she's happy and she is very well loved.