Hypoplastic Left Heart Syndrome

This is baby B's “heart defect” to which I have referred but had not yet identified.

As you might recall, when we went for our anatomy scan at 19w2d, we were told that there might be a problem with our little girl, baby B’s, heart. The ultrasound tech said she couldn’t get a good picture of the left side. One of the MFMs, Dr. G, came in and agreed that the left side could not be seen well – but, baby B was always smaller, always measuring a little behind baby A. So maybe it was just too early to get a really good look of her heart. Dr. G. told us to come back in a week and retest. Hopefully everything would be fine. If not - to a pediatric cardiologist we go.

That night my OB called and told me to go to the hospital. I had no idea everything was falling apart. All I could think of, all I could focus on or bargain for – was that my little girl B’s heart be okay.

When I was in the hospital, after I delivered our babies, my "head" MFM, Dr. C, requested an autopsy to look at baby B's heart. So, was it related?, I asked. Did my HELLP Syndrome have anything to do with her heart defect? No, he said. Nothing at all.

You mean there were *more* cards stacked against me and my babies?! I cried. It's not enough that my body apparently *hates* to be pregnant??

Yes, it seems so, he said. Double whammy.

(Incidentally, I received this same response when I asked my RE if my clotting disorders that led to my HELLP caused my infertility. She said no. Double whammy?, I asked. Yes, she responded. You’re just my unluckiest patient. So make it a tipple whammy: IF, clotting disorders/HELLP Syndrome, and heart defects. But I digress.)

The autopsy results were inconclusive. I was crushed. I needed answers. So I asked for the report from the anatomy scan. And there I saw it: “Probable HLHS.” Hypoplastic Left Heart Syndrome.

I spent the rest of the afternoon glued to my computer:

Hypoplastic Left Heart Syndrome (HLHS) is one of the most complex cardiac defects seen in the newborn and remains probably the most challenging to manage of all congenital heart defects. In a child with HLHS, all of the structures on the left side of the heart (the side which receives oxygen-rich blood from the lungs and pumps it out to the body), including the aorta, are severely underdeveloped. Infants with HLHS develop life-threatening shock when the ducutus arteriosus begins to close, typically within the first hours or days of life.

The preferred treatment is a heart transplant within the first 24 hours of birth. Since often there are not organs available to transplant, the most common treatment is a series of three reconstructive operations, the first performed within the first week, the second during the first six months, and the third within the first two to three years.

When we went for our follow-up with Dr. C, I asked him – So really, doctor. What do you think was going on with her heart? He started speaking slowly: Well, Dr. G is one of the best in the business. She knows how to read ultrasounds, and she knows how to find what she’s looking for. . .

Translation: what she was looking for – the left side of my baby’s heart – essentially wasn’t there. Hypoplastic Left Heart Syndrome.

Rate of recurrence: 5%. Maybe as high as 25%. One study claims a 13% recurrance risk and a 22% chance of some other cardiovascular malformation.

Nuts.

I tell you this, dear friends, so that, should I find myself pregnant again one day down the road, and I come to you with my anxiety and fear, you will know all its many sources. Fear that my body is failing my child. Fear that my child's body is failing him or her. A tripple whammy. So many insults added to injury. It's all a little hard to get my head around.

I feel valid in my anxiety; it's not unfounded. The science - the numbers - are very, very real. And only my baseless belief that we should now be immune from all bad things keeps me hopeful that we will, for the first time, fall on the right side of the odds.

A Mother

Here in the babyloss community we call ourselves “mothers.” And I suppose it’s true. I have been forever changed. I have carried two perfect examples of life inside of me. I loved them with every ounce of my being and in ways I never before understood or thought possible. I gave birth to two perfect little babies, and will forever carry the weight of their absence. My inability to protect them shattered me to the core.

And I can remind myself of these things all day long. But I don’t feel like a mother.

There’s no nursery in my house. There aren’t any bottles or sippy cups in my cupboards. We don’t have car seats in our cars. We sleep in and stay up late. And we save money for fertility treatments rather than college. I sing songs to my dogs, rather than a child (embarrassing but true), and I rock – well, I don’t rock at all. I don't bandage knees or break up fights. I don't cut the crust off bread or cut grapes into quarters. I'm not sending new photographs to doting grandparents. My arms are empty. Painfully, obviously, empty.

And the world certainly doesn’t view me as a mother. Not ever, but particularly not on this coming Sunday. No one will be sending me a card, taking me to lunch, or handing me a gift. There would be no corsage for me at church.

Sometimes I want to scream at the world and convince it of my mother-ness. But most of the time I just feel like a fraud. I want to embrace Mothers' Day in a bittersweet way: bitter because my little boy and girl - my son and my daughter - are not here with me; sweet because I am still their mother.

But if I am a mother it is only in an intangible, emotional sense. (And, I suppose, in a physical sense too, if you consider my reshaped body.) The practical side of me doesn't even remotely feel like a mother. Which, I suppose, is the real source of the heartache after all.