Many of you are aware that on March 11, 2010 - during a routine mid-term pregnancy ultrasound - our unborn baby boy was diagnosed with Hypoplastic Left Heart Syndrome (HLHS).
As the sonographer moved regularly through the body systems - checking out the head size, brain, etc. - I noticed that he skipped the heart. In addition, I noticed that he was conducting a much more thorough ultrasound than I have had with my other pregnancies. It was Jay Thomas the same sonographer who had done our other ultrasounds, but this time there was something different. He made many measurements of all of the long bones; he repeatedly looked at the hands, feet, and the face/lip. Everything even the spine looked fine to me.
Then after almost an hour into the ultrasound he finally moved to the heart. Immediately it looked strange to me. I thought maybe the baby was just in a strange position and so I was not able to orient myself with what the ultrasound was showing. Jay had me roll on my side and we tried to get some different views. I knew something was wrong because Jay's normal smooth continuous way of conversation had ceased. As the three of us sat in the dim room staring at the ultrasound screen I could tell that the baby's heart was not normal. I could only see 3 chambers. The heart looked a little twisted or bent as it wrapped around the position where the missing chamber should be. Even Michael with no medical knowledge started getting anxious and asking questions that the sonographer was hesitant to answer. He could really only disclose to us that the heart had a severe malformation. I asked, where is the left atrium? Where is the left ventricle? Why does the septum look bent? Is the aorta too narrow/small? He vaguely answered my questions, and stated that he would immediately have the radiologist read the ultrasound and contact Dr. Nance (our O.B.) and then Dr. Nance would discuss it with us.
I barely held it together during the last of the ultrasound. I could not get my clothing straightened around fast enough so that I could get out of that horrible little room. I felt like my chest was going to explode. Even though we did not have a diagnosis, I had enough information to know that it was extremely bad. In less than one hour our lives had been changed forever. We walked into that ultrasound room with the vision and excitement of receiving a glimpse of our little boy, and we left with broken hearts.
I only made it to the waiting room when I could not contain my grief any longer. I burst into tears and went straight to the car. There are very few times in my life that I have felt the heavy and sudden feeling of grief like that day. I cried all the way home. Micheal kept reassuring me that everything was going to be fine. I think we both knew that everything would be fine, but what does fine mean?
I did not return to classes that day. I immediately went home and started researching congenital heart defects, and I dug out my pediatric nursing books. Within hours I had it narrowed down to only one defect, Hypoplastic Left Heart Syndrome. When Michael returned home that evening I emotionally discussed what I thought the diagnosis would be. At first he was in denial, but when he started doing a little research himself he soon found that I was probably right.
The next day we received a phone call from Dr. Nance. Again we did not receive any helpful information but was told that we would need to meet with a perinatologist at Utah Valley Regional Medical Center. I could not wait to get an appointment and immediately I started making phone calls. The next Tuesday, Michael was supposed to leave to go out of the country for 8 days. I wanted a diagnosis before he left and knew that we would still need to have a fetal echocardiogram at Primary Children's Hospital and talk to a pediatric cardiologist before they would give us the diagnosis.
After lots of phone calls and harassing the secretary and RN in the Maternal Fetal Medicine Department at Utah Valley Regional Medical Center, they pulled some strings for us and we got an appointment on the next Monday (the day before Michael was suppose to leave) with the pediatric cardiologist and to have a fetal echo.
It was a very long weekend. I spent almost all of my time on the internet reading medical journals, medical articles, information pamphlets about congenial heart defects, and finding websites that had useful information about heart conditions. We were sure that our baby had HLHS.
When we arrived at Primary Children's Hospital on Monday they were immediately ready to conduct the fetal echo. After the tech was finished she got the pediatric cardiologist to take a look and then they asked us to move to a different room. Yes, you know what room I am talking about, "the quiet room." That’s the room the medical professionals always take the family to when they have bad news to give them. Even though we knew what they were going to tell us, I still could not help thinking, "is this really happening to me?" Dr. Menon was polite and informative and gave us the diagnosis we had expected. Our baby has Hypoplastic Left Heart Syndrome. The social worker was also there to help us start the grieving process, but we had already begun that on the previous Thursday.
We were prepared for all of the information that they gave us. I will include information that I found online about HLHS so that if you’re interested you can understand the options that we have and the choice we must make.
Basically we have 4 choices:
1) Terminate the pregnancy - of course we would not choose that, there is no risk to my life.
2) Comfort Care - deliver the baby at full term and then let him pass away.
3) The Norwood Procedure - a very extensive open heart surgery that is only palliative not corrective. In addition the baby would need at least 2 other surgeries following this one during the first few years of life.
4) Heart Transplant - not really an option because there is not really any newborn hearts available for transplant.
Read the information under Hypoplastic Left Heart Syndrome under links...