The First Signs
My experience with a congenital heart defect is not exclusively my own experience, but it is probably as personal as it can get. Born at just 34 weeks at a respectable 5 pounds 4 ounces, my son, David, was a healthy baby albeit an itty bitty one. At 9 months, we decided to move forward with surgery to correct a miniscule urological condition that is common among preemies. The surgery progressed in a perfectly normal manner, even with a slight re-bleed from the incision. In fact, David had breezed through the surgery with little more than some agitation coming out of anesthesia. We thought that everything was fine and that we were on the road to recovery.
On the evening after the surgery, I noticed that David’s lips, fingertips, and toes had a blue hue to them. I could attribute it to being cold, but he was fully dressed and in a well-heated room. In fact, I remember clearly that his hands were sweaty. Within 2 hours, he began to spike a fever – almost 105°. I was already waiting for the urologist to call me back about the blue spell, but given the fever I felt it was important to just get him to the Emergency room. After 12 hours of monitoring, we were sent home with no answers and instructions to follow up with the pediatrician. Urology felt that it was some sort of underlying virus and not their concern, while his pediatrician felt that it was for urology to follow up with considering the time line. Needless to say, we were hitting road blocks at every turn. Within two weeks after his surgery, David had three more blue spells and two more major fevers. At this point, I knew that I had to advocate for my child since our concerns were not being addressed. As a parent, you are the only voice that your child has – David’s was not being heard.
Finding the Right Path
I made the decision to switch to a pediatrician that was directly associated with Children’s Hospital of Philadelphia. I can’t say that it was a quick road to a diagnosis but after a sleep study, a pH probe, a sweat test for Cystic Fibrosis, and finally a cardiac work up, we had our answer. David suffered from a Bicuspid Aortic Valve. We learned that his blue spells, otherwise known as cyanosis, were a direct symptom to his heart compensating for the defective valve. After a month long Holter Monitor testing, it was determined that his cyanotic episodes correlated with the Holter readings and cardiac activity.
Naturally, this news came with relief and anguish for my family and me. On one hand, it was a clear direction. We had an answer and could subsequently develop a plan. On the other hand, it was not an easy fix; there was no magic pill or simple approach to eliminating this defect. Yes, we had a diagnosis, but now what?
What Comes Next?
Of course in the beginning, we prepared for the worst. We expected to need valve replacement via open heart surgery with extensive recovery. We prepared for repeat surgeries as David grew to compensate for the artificial valve. What we ended up with was surprisingly and gratefully less dramatic. David’s valve was indeed bicuspid, but over two years of monitoring, his body grew to adapt around the incompetency. In fact, he presented no hardening of the valve without any regurgitation of oxygen-rich blood (two common effects from a Bicuspid Aortic Valve). We are now at year six of following his heart condition and the only effects we have seen have been a slight widening of the valve opening. His defect is otherwise growing with him completely and is functioning as a complete unit.
This isn’t to say that it isn’t without precaution. Because the pulmonary and cardiac systems are so intertwined, David did undergo a tonsillectomy with adenoid removal and a complete mesh airway lift to increase oxygen flow within his body. He wears a heart guard for almost all physical activities to decrease any possibility of injury that could affect his heart, heart rate, or sternum. David struggles with pulmonary issues and has developed pneumonia on multiple occasions which is a direct result from his heart condition. That said, his condition is still completely manageable overall.
Coping
Having a child diagnosed with a congenital heart defect changes a lot of things. First, it’s the initial slap in the face of mortality for your child. You see their lives changing or worse. Second, any other children that you have will be affected. My second son has already been to a cardiologist, has had an EKG, Ultrasound, and Echo of the heart. Thankfully, his heart is “normal” by textbook standards but the experience is still there. There is the unsurmountable possibility that any children you have may have a congenital heart defect, and you may never know where it actually came from. Coping was tricky. We were fortunate that my son’s body has adapted so well, especially when we met others that have struggled with the same condition. I felt guilty being so consumed with fear and worry for my son when I knew for others it was devastatingly worse. But I also knew that my fears and worry came from a place of complete love for my child.
Our answer to coping emotionally came from support from the CHF community. We started Team David for the Congenital Heart Walk and helped raise money to support those that were battling congenital heart disease. It was our way of giving back for our blessing of a manageable defect. Having a congenital heart defect is not a death sentence, but knowing that your child will likely need to confront the disease is a terrifying feeling. I was fortunate to have a fantastic medical team and a son that was strong enough both emotionally and physically to keep moving forward.
