It's taken me some time to finally write this up, not because it was difficult to write (a month or so ago that might have been the case), but because I wanted to get it down correctly.
Rather than beat around the bush, I'll just start with the most critical issue and work backwards.
Our daughter Lyra has a congenital heart defect called Hypoplastic Left Heart Syndrome.
What is Hypoplastic Left Heart Syndrome?
HLHS is when the Left side of the heart, predominately the left ventricle, never fully develops. If you were to look at a diagram of a human heart, her's has a normal sized right aorta and ventricle, but the left side is very small and unable to operate as a pump. We learned that this defect has no effect on the baby because they get their oxygenated blood from the mother's placenta. So, one at least marginally reasurring aspect of this is that the baby is perfectly fine and will develop normally. It's only after they're born when the problem affects them.
It's a hard thing to be told that your unborn child has a serious health problem. And as these things tend to come as a complete surprise, it's a very hard thing to accept.
The first indication that anything was wrong came during the second ultrasound. The doctor felt that there was the possibility that Maren had an elevated level of amniotic fluid and recommended a level 3 ultrasound, a more detailed test that can take more accurate measurements. In retrospect it seems clear to us that they had probably observed something wrong with the heart at this time and wanted a more definite diagnosis.
Of course we were not aware of this and went to the level 3 ultrasound with some concern, but we weren't really too worried. Even during the ultrasound things seemed to be going well. The nurse doing the test kept showing us good signs, like the right number of fingers and toes, the fact that she was moving her hands, etc. However, when the Doctor came in to look, I sort of knew something was wrong because the originally chatty nurse suddenly became quiet and the Doc didn't say much at all after his initial greeting. He then sat down and began peering intently into the ultrasound monitor.
It was after this round of testing when we first heard about HLHS. We were referred to Duke hospital to have a fetal echocardiogram performed. This was done essentially to corroborate the diagnosis, which it did.
We spoke with the pediatric cardiologist from Duke about our options, of which there were really only two, a heart transplant or a three-stage surgical procedure. After talking with the cardiologist we had the chance to talk to the chief of pediatric cardiac surgury at Duke (likely the surgeon who would be perfoming any operation on Lyra). We found out then that the heart transplant isn't recommended much any more, mostly due to the lack of heart donors and also I believe the much improved success rate of the staged surgury.
Instead of trying to explain everything about the surgury, I'll try to hit the main points and post some links to more detailed information below. I'll also post them on the front page.
Stage 1 is called the Norwood procedure and involves reconfiguring the heart to use the right side of the heart to pump blood to the body and insert a shunt to provide blood flow to the lungs. This is the most complicated surgury and has the greatest risk involved.
Stage 2 is done to remove the shunt and reconfigure a vein and artery for the same purpose, i.e. blood flow to the lungs.
Stage 3 is called the Modified Fontan procedure. The first two surguries involve making sure blood flows to the lungs and allowing mixed blood (oxygenated and deoxygenated) to flow through the body. The fontan prodedure is yet another reconfiguration that will separate oxygenated and deoxegenated blood flows.
While Maren and I have our apprehensions at times, we're hopeful that these surguries will allow Lyra to have a normal life. It's amazing to think that only 20-25 years ago, such a defect was rarely detected and was always lethal. This defect is also not as rare as one might think, almost 1 in every hundred babies are diagnosed with this problem. We're somewhat lucky to be living this close to a hospital that has significant experience performing these surguries.
Maren and I welcome any messages of support, so please feel free to email us at:
Maren
Links:
There's a link on the front page in the first paragraph for basic information. It has a great booklet that you can view online or download as a PDF with all the detailed info on the Syndrome and details on each surgical stage.
Royal Children's Hospital Cardiology Department:
This site is a bit cartoonish, but it also has a decent booklet about the defect and good descriptions of the surguries.
This was a Dateline episode that covered a family with a baby that had the same problem. It's a bit hard to read knowing that you have to go through the same thing, but it helps a little knowing that others have gotten through this.