Julia is our sweet 15-week-old daughter. She loves to smile and gurgle at mommy and daddy, and she has a newfound fascination with "The Very Hungry Caterpillar" stuffed animal. She is sweet and calm, and she rarely cries in a manner typical of a 4-month-old. She does use increasingly loud vocalizations to get our attention, and we are loving the way that she is communicating with us. She has just begun occupational therapy and is working on skills like holding her head up and feeding.
Julia also has Down Syndrome (DS). She has a heart defect that is common for children with DS, called a complete Atrioventricular Septal Defect (AVSD). Roughly 20% of children with DS have this type of defect. When Julia's body was forming, her heart did not form correctly. Rather than having 4 separate chambers in her heart, Julia has essentially one large chamber. The walls that are supposed to separate atria and ventricles are incomplete, and instead of two valves regulating blood flow between atria and ventricles, she has only one.
Normal Heart
Julia's VSD (the ventricular, or lower, part of the defect) has been described as "massive." Her heart is ever so slightly unbalanced so that her left side is a bit stronger than her right. All other components of her heart (arteries, veins, structures) are in perfect condition...and we should know, because this girl has already had lots of tests and imaging done to determine what her surgical needs are!
Complete AVSD
Repaired AVSD
Problems Julia has had resulting (at least in part) from this heart defect included difficulty feeding, difficulty rousing, and weakness. The first month of Julia's life was very, very hard, because feeding her was so challenging. She appeared to be suffering, and she probably was. She still struggles with feeding in some ways, but she is definitely getting enough calories, though she is only 10lbs. What many of the other "heart moms" tell me is that these children are like different babies once they have this heart surgery. Suddenly, all those calories that were sustaining a failing heart are now able to be used for more fun activities, such as movement, interaction, and getting nice and fat!
The early days...in the NICU
Nationwide Children's Hospital, in Columbus, OH, has the #5 pediatric cardiology program in the nation. Julia's surgeon, Dr. Mark Galantowicz (google him!), is a pioneer in many different types of pediatric heart procedures, including "bloodless" heart surgeries, where no blood transfusions are required. He flies all over the world giving talks and performing procedures on children who greatly need them. We feel incredibly blessed that all we had to do to get this surgeon to operate on our daughter was...ask. Just ask. Our cardiologist assured us, "This surgery is the bread and butter of pediatric cardiothoracic surgeries." In other words, it's very common and straightforward, and most of the time, outcomes are great.
Simply the sweetest (15 weeks old)
There are several possible complications, but most of these are pretty unlikely. She could require more surgeries if the repair doesn't go well and she still has a lot of leakage from her heart. This isn't terribly likely for Julia, as she has very minimal leakage right now. She could also require a pacemaker if the electrical rhythm of her heart is disrupted during the surgery. The greatest risk for Julia is actually something that would be managed with medication. Because she is doing so well right now, we know that her body has compensated for the defect by increasing the lung-artery pressure. In fact, her heart defect is so large and her lung-artery pressure is so high that she doesn't have an audible murmur - even the cardiologist can't hear it. This is good right now, but when the defect is repaired, she will likely have to be on medications to help her body get back to normal.
She will be on several medications for about a year, and eventually we will only see the cardiologist once annually.
Julia's surgery is scheduled for Wednesday, November 6. We will likely be in the hospital for about a week as her body recovers. These kids recover amazingly quickly, though weeks 2-3 at home are the most crucial in terms of her risk of infection. Because of this, we will likely be quarantined at home for about 4 weeks to avoid infection.
