HLHS (Day #8 of A to Z Blog Challenge)

HLHS (Hypoplastic Left Heart Syndrome)

I have talked about our little S a lot lately. It has been four years since he died. Just about five years ago, unable to sleep at 5 am, I took a home pregnancy test that came up positive immediately (no waiting for a minute or two. It was immediate.) I was so, so excited. We'd conceived the very first month we tried. I'd felt (as did my husband) sure that it would take at least a couple of months of trying.

I should have been taking more folic acid . . . could have been off a very small dose of an SSRI for anxiety . . . lots of woulds, shoulds, and coulds.

At our 20 week ultrasound, the one most people think of as the "gender ultrasound" (and guess what? That is NOT the purpose of the Level II ultrasound, people!), as my husband and I were discussing who the baby looked like - his head shape took after my side of the family - the ultrasound tech got very quiet and then very concerned and brought in the perinatologist. There were a few problems . . . 2 vessel cord was the first thing, and then it was his heart. She couldn't see it all. It turns out that is really wasn't all there.

The perinatologist originally (and rather unkindly) thought it was Cushion Syndrome, and that our baby probably had Down Syndrome, too. He recommended amniocentesis asap.

Instead, we waited, trying to digest what was going on. We had to wait an entire week (it seemed an eternity at the time) to get a fetal echocardiogram with a fetal cardiology specialist.

She didn't believe that S. had Down Syndrome . . . because S. did not have Cushion Syndrome. He had, instead, Hypoplastic Left Heart Syndrome with Double Outlet Right Ventricle. She told us all of our options - a series of surgeries to re-route the blood flow after he was born, carry him full term and then basically do nothing, or if we wanted to end the pregnancy, we needed to decide soon, because he was already 21 weeks along by that point.

Our first baby. We were so excited, and now we had to truly start being parents. We had big - no - huge decisions to make concerning his future.

Here is description (from http://www.thic.com/hypoleft.htm)- there is also a diagram there:

"Hypoplastic left heart syndrome refers to underdevelopment of the left side of the heart. This syndrome may include:

A very small (hypoplastic) left ventricle (the lower chamber which normally pumps blood out to the body): It is too small to pump enough blood throughout the body.

Small aorta: This is the major blood vessel from the left ventricle to the body.

Aortic valve atresia (absence): This valve normally opens and closes to let blood flow from the left ventricle to the aorta. When atresia is present, there is no connection between the left ventricle and aorta, and no forward blood flow.

Mitral valve stenosis or atresia: This valve normally opens and closes to let blood flow between the left atrium and left ventricle. Stenosis causes little blood flow; atresia causes no blood flow. Either atresia or stenosis may be present.

Your physician can explain which defects are present in your child.

This very serious combination of heart defects results in little red blood (blood high in oxygen) getting to the body. Two normal connections that infants are born with, provide some blood flow. These connections allow the infant to appear healthy at birth. However, as these connections begin to close, the infant quickly becomes critically ill. Surgery for this defect may be performed in stages or cardiac transplantation may be considered. Successful surgery has only been accomplished in the last few years and long term results remain unknown."

That last sentence is what bothered us the most - the long term results. My husband was worried that maybe our son (we did learn he was definitely a boy at that 20 week appointment) would die at age five, at age ten, age 20; that he would not have a good quality of life. I just wished we could go back and start again. Lyrics from "Jesus Christ Superstar" kept running through my head: "This was unexpected / What do I do now? / Oh, could we start again, please?"

Ultimately, we continued, and the plan was that S would have his first surgery (Norwood Sano) soon after he was born. To make a long story short, he had it at eight days old (S surprised people - they thought he was behind in growth - and was 7 lbs 10 oz!), and the surgeon made an unwelcome and rather shocking discovery. He couldn't see some important veins that should have gone from his heart to his right lung. Still, the surgeon seemed very up beat about S's chances.

S never made it home from the hospital. They kept finding thing after thing, and then he couldn't stay off of the ventilator (it turns out that he was indeed missing the veins, and because of that, his right lung was hypoplastic - underdeveloped - and his poor little heart was shifting over to the right). For a while they thought maybe he had some form of congenital diaphramatic hernia, but he didn't. They kept saying that he had something genetic . . . but the tests were done, and he had something, but they didn't find anything really specific or identifiable on the the genetic tests. His collection of defects does not match any known syndrome, either.

With conceiving our second son (who is a very active and very healthy 2 year old), I was not on an SSRI, I was on a prescription pre-natal vitamin, and taking more folic acid beyond that - did you know that folic acid can help prevent congenital heart defects? - and was just very hopeful that he would be fine. We had a fetal echo done at 16 weeks. The first thing I asked the tech was whether there was a three-vessel cord! She was surprised (we lived in a new location, so new people for everything) that I asked such a question, but I explained why I asked. He had a normal three-vessel cord, and then we saw (after S, I knew how to look at ultrasounds, echoes, images pretty darn well myself - and know what things are and are not supposed to look like) that he had two working ventricles of his heart. I cried with happiness. Everything else (other organs) appeared to be intact and working, too! Of course, we couldn't know about his lungs until he was born, but since everything else was looking very good, we just had to have faith that his lungs would be fine.

Now, whenever I find out someone is expecting, I don't ask if it is a boy or a girl first. I ask if the baby is healthy. It is really, truly the most important thing. And so many people take it for granted. I also mourned for my loss of innocence. I would so love to be ignorant of all that could go wrong during pregnancy, at birth, after birth, etc. Sometimes I just want that innocence back.