Jeff and I have been married for almost six years. After two years of waiting for a baby and a little help from fertility medication we found out I was pregnant in January 2013.
On Monday, May 6 (19 weeks) we went in to have our anatomy scan and find out if we were expecting a baby boy or baby girl. We found out that we were expecting a BOY, but the sonographer was having a hard time seeing his heart. She asked us to come back a week later for another try. I was a tiny bit worried, but was easily reassured that everything was normal and this happens all the time.
Monday, May 13 (20 weeks) we went back for more ultrasound pictures. She was very quiet and focused on his heart. After about 30 minutes she went and got another sonographer who looked at his heart some more and took several pictures. They didn't/couldn't tell us anything. As we left they told us they would send the pictures to my doctor and "we'll go from there." I left this appointment extremely worried that something was really wrong.
After two extremely long days of waiting, worrying, and speculating, I got a call from my doctor on Wednesday, May 15. She said that what they saw was an "underdeveloped left ventricle" in our baby's heart. She was going to refer us to a maternal fetal specialist for more care. I called Jeff, then called their office right away to schedule an appointment. The waiting/worrying/speculating was killing me. We scheduled an appointment for the next morning. I googled (I know, so dangerous...) "underdeveloped left ventricle" and read all I could find. A diagnosis called Hypoplastic Left Heart Syndrome (HLHS) kept coming up. I wondered if that was what we were really dealing with and if so, I learned it was a very serious congenital heart defect that would require lots of surgeries and a bumpy road ahead. We were scared. Jeff and I got priesthood blessings from my family that night and had my parents take us to the appointment the next morning.
Thursday, May 16 we met with a maternal fetal specialist who did another ultrasound and confirmed the diagnosis of HLHS. He gave us three options for the pregnancy: to terminate the pregnancy, to carry the baby to term and give him comfort care after he is born, or to deliver the baby and go through with the three stage open-heart surgeries and bumpy road ahead. Since he specializes in pregnancy, he couldn't answer many of our questions about the baby's life after birth. He referred us to a pediatric cardiologist for more care. He was able to get us in to see Dr. B the next morning.
Friday, May 17 we saw Dr. B. They did a high-powered ultrasound called a fetal echocardiogram where they could not only see his heart, but could determine which direction blood was flowing and even measure blood pressure in specific parts of the heart. After the fetal-echo, Dr. B spent over an hour talking us through the diagnosis of HLHS, giving us a crash course in heart anatomy, and answering the many, many questions we had. We also learned at this appointment that our baby had Total Anomalous Pulmonary Venus Return (TAPVR), which is another complication with the way his pulmonary veins connected to his heart. We planned to see him in another month.
Over the next several weeks we educated ourselves about all things HLHS. We came up with many more questions and felt quite a bit of reassurance that things would probably be okay. We found other families who had been though this process. We learned that although the road would be bumpy, about 80% of babies born with HLHS survive to at least the age of 20 or so. We had to change our plans of having a "normal" baby to having a baby with a severe heart defect.
At our appointment with Dr. B on July 22 (30 weeks), we learned of another complication our baby had. They noticed on his fetal echo that there was some hypertension in his left atrium. This was caused by blood flowing to his left atrium and not having a good way to get out. There were a couple things causing this problem. First, he does not have an Atrial Septal Defect (ASD), which is a hole between the upper chambers of his heart. 99% of HLHS babies have this, but our baby does not. Second, he has an anomalous "decompressing vein" going from his left atrium to his Superior Vena Cava which is obstructed and not allowing enough blood to flow through it. These complications were causing blood to flow backwards to his lungs and cause damage. This was a much more serious diagnosis with a much worse prognosis. Dr. B could not answer many questions about statistics or prognosis at this point because it is such a rare case.
Over the next few weeks we learned more about HLHS, this time with the added complication of an Intact Atrial Septum (IAS). There was not much research to be found, but we knew it was much more serious than what we were dealing with before.
On August 13 (33 weeks) we went back for another fetal echo. They took some more measurements and Dr. B talked us through some research he had gathered about HLHS and IAS. We found out the prognosis for our baby boy was not good. They gave us a 5-10% chance that he would live "long term." "Long term" meaning 5+ years. Depending on how healthy or sick he is when he is born will determine what interventions they can try after birth. We really don't know what we can expect as far as his life here on earth. We may get to spend just an hour with him, or it could be a few months. We are trying to prepare ourselves for the worst, but hope for the best.
Thankfully, we know that we are an eternal family and that no matter what happens, we will be able to have Ethan with us after this life. We have such great support from family and friends that helps us to keep going every day.
We plan to deliver Ethan on September 30, 2013 and cherish every precious moment we have with him here on earth.