I HEART SOPHIA

Sophia Alexandra Reynolds was born during the late afternoon of October 27th, 2004.

Though Patty had some slight complications with her pregnancy, Sophia was born with no discernible defects. And was given high marks on her assessments.

Soon after, we were moved from the delivery room to the room where mother and baby would stay while Patty recovered from her labor. Within a couple of hours, we noticed Sophia was having trouble breathing and it was suspected she had some sort of obstruction in her airway left over from delivery. Her color was slightly bluish and we were of course concerned but assumed this to be an easily remedied situation. She was eventually moved from our room to the nursery for observation where she continued to have problems and was eventually sent down the hall to the intensive care nursery for evaluation.

Obviously by this time, which was getting late into the evening, we began to worry something was wrong. By around midnight, we met with the intensive care doctor. He explained that he suspected a heart defect, specifically a pulmonary artery defect, but this would have to be confirmed by an echo-cardiogram. We were stunned. Just hours earlier, our main concern was how we were going to care for our healthy baby girl. Now, our whole world had been shattered and we had no idea how the pieces were going to land.

A cardiologist, Martin O’Laughlin MD, was called in to do the echo. And in the middle of the night (3am?), he came into our room to give us the results. The NICU doctors original diagnosis was off a little bit, but Sophia did have a pulmonary heart defect. This would be the first time of many the words Tetralogy of Fallot would be spoken in relation to our precious daughter. Plans were made to move her to the local children’s hospital the following morning.

Then we finally got to see her again. She looked so different than she had just hours earlier. She was in an oxygen tent and grunting with every breath she drew. It was so devastating. Here was our first and only baby and she was the most beautiful thing I had ever seen. But, she was struggling so hard to hang on to life. Twenty-four hours earlier we had never even thought the words, congenital heart defect. Yet, here we were.

We spent several weeks in the NICU at the children’s hospital and eventually a heart catheterization was done. The original diagnosis was confirmed but one additional detail was added. Sophia’s pulmonary artery was only partially formed and, because of this, it did not connect to her heart. Instead, there were many small vessels from the aorta that were transporting blood to the lungs for oxygenation. This defect was called pulmonary atresia with multiple aortic-pulmonary collateral arteries (MAPCAs).

We were later sent home for a few weeks and ended up having her first surgery after her cardiologist sent her from his office to the emergency room.

I won’t continue with all the details. But, I thought it was important to share the way we found out about Sophia’s CHD. Which, again, was Tetralogy of Fallot with Pulmonary Trunk Atresia and MAPCAs. Sophia’s aorta also arches opposite the direction it should.
Tetralogy of Fallot consists of a ventricular septal defect with the Aorta centered over the hole between the two ventricles, an overly muscular right ventricle, and usually a narrow or stenotic pulmonary artery and valve. But in Sophia’s case, she had no pulmonary artery where it would normally be narrowed.

The first surgery Sophia had was called the Blalock-Taussig (BT) Shunt. It happens to be the first heart surgery ever invented. It involves placing a tiny gortex conduit from the aorta to the pulmonary arteries. Thus giving the body a way to return blood to the lungs for oxygenation.

Later, Sophia had her second surgery. This would be her first open-heart surgery. During this surgery, the shunt was removed. Sophia’s heart was stopped and a donor tissue conduit and valve was placed from the right ventricle to the pulmonary arteries. During the surgery, the surgeon carefully gathered as many of the MAPCAs together and joined them to the pulmonary arteries. Many of these collaterals were smaller than a millimeter in diameter. This is called an unifocalization.
Sophia eventually developed a pseudo-aneurysm at the point the conduit is joined to the heart. This required a third surgery (second open-heart) that was nearly a repeat of the previous surgery minus the unifocalization.

As of now, she has not had any more heart surgeries.

It should be noted, Sophia’s defect is caused by a genetic syndrome called DiGeorge Syndrome. This syndrome includes a myriad of defects and delays. At this point, aside from the heart defect, Sophia has only shown evidence of feeding/speech problems and some gross motor delays.

In summary, Sophia’s heart defect is partially repaired. She will require another surgery in the near future to close her VSD (ventricular septal defect). She has some speech delays and is behind with her gross motor development.

Sophia regularly sees a cardiologist, two pediatricians, a physical therapist, a feeding/speech therapist, an occupational therapist, a special instructor and several other doctors as needs arise. She takes several medications per day and takes most of her fluids through a feeding tube. This can be challenging to us as parents and I am sure it is no picnic for Sophia. But Sophia is an exceptionally bright and happy little girl. And defects or not, I cannot imagine wanting her to be any different than she is.

You can read much more about the details of all these procedures and challenges in the older posts of this blog. There is a comprehensive back story regarding Sophia’s diagnosis and early challenges starting at the bottom of this page.

You can also view slideshow videos of her first and second years on youtube.

The blog is here.