Trying to hold ourselves together and find our new normal after loosing our precious twin babies at 20 weeks to HELLP Syndrome.
Baby Boy A and Baby Girl B, conceived after 20 months and IUI plus injectibles; born still on November 12, 2008.

A blog about pregnancy loss and infertility. And whatever comes next. A blog about hanging on together and holding each other up.


Tuesday, May 26, 2009

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.

23 comments:

Courtney said...

Oh my dear friend I so wish you didn't have anything else on your plate. You're right, we should be immune from all other terrible things in our lives especially when it comes to pregnancy and our children.

*hugs*

Busted said...

Things really do seem utterly unfair sometimes. I hate that you have something else about which to worry. And even if you had nothing working against you scientifically or statistically, we'd understand your anxiety and support you through it. (((HUGS)))

Megan said...

sometimes it just piles on, doesn't it? hoping everythign will go right next time after everything went wrong...

Mommy (You can call me OM) said...

Oh, Bluebird. I think you have every right and every reason to feel anxious should you fall pregnant again (I think I've been reading too many of our Aussie friends' sites!).

I think that if you (and all of us) try to take one thing at a time, just as we take one day at a time, it might not all seem so overwhelming. That's so much easier said than done, I know.

Please know that we'll be here, no matter what.

Peace, my friend.

Donna said...

I really, really wish there was a "get out of bad things free" card that could be played by all of us who have already been through more than our share of heartache and pain.

When you are pregnant again we will all be here to cheer you on and pick you up when you are down.

The granddaughter of a co-worker was born with what sounds like a similar heart defect. She had several surgeries as a baby. She and her family have been through a lot but she's now at least 10 years old and doing great. (not sure if that's what you want to hear or not - but at sometimes it's good to hear about a success story)

Mimi said...

Let me tell you something. When you do become pregnant again, and you will! I will be here every step of the way during your fears and your joys. You will be a mommy (you alredy are) I'm sure you know what I mean. You will have a healthy baby and you will be such a wonderful mommy. Everything will work out and your happiness will return 100% again.

Thinking of you always,
Kami

just me, dawn said...

I will be here praying for your (and all of our) immunity. and when you are pregnant again, I will be here to rejoice and worry with you.

JenJen said...

I think any anxiety you feel WHEN you get pregnant again is not irrational, but one based on life experience. It truly is very sad that baby b had so much stacked against her...You didn't fail her, and her body didn't fail her...God just makes us and thats all there is.

Lea said...

Bluebird, you have every right and reason to be worried and anxious. You have dealt and continue to deal with so much.

We will all be here for you.. to support you when you get pregnant again.

xo

Ya Chun said...

holy triple freakout.

is hlhs linked to twins? or can it happen in singlets too?

Michelle said...

You really should be immune from now on! I really wish that is how it all worked. I think all anxiety you feel when you fall pregnant again will be completely justified, and we will all be here to support you when it happens.

Katie said...

It seems like sometimes it just never ends. I'm so sorry you have all this on your plate. I wish I could take some of it away for you.

Shanny said...

My jaw just dropped at the insanity of it all. It is so not fair, none of it, its just too much and I'm sorry you were so unlucky. I wish there was more I could do for you than to pray for a luckier brighter future, but since that's all I have that's what I'm going to do. It tripple freaking sucks =(

Mrs. Spit said...

Oh Bluebird, there are no words, are there?

Hugs. Big Hugs.

Brenna said...

Sheesh bluebird, I'm so sorry to hear this. Enough should be enough, already, shouldn't it? I agree with Donna, we've earned our 'get out of bad stuff free' cards and shouldn't have to fret over potential future bad stuff. But of course we do...we're human. And all of us humans who love you will be right here to listen to any venting and anxiety you need to share, when the time comes.
xxoo

Jacinta said...

Sweet Bluebird, I was so sad reading this post.

I will be hoping with all my heart that you fall on the right side of the numbers.

Heather said...

I agree- I think we should all be spared from future horrors after going through what we went through.

I'm so sorry you have all of this to deal with at once.

Erica said...

Hugs to you! I do know how you feel mama and my heart aches for you. I didn't experience both at the same time- but I have separately- as you know. Know that I am here if you ever need to vent!

Another Dreamer said...

Sorry sweetie. Big (*HUGS*) to you. I never would have questioned your worries, I get it. I really do.

niobe said...

Sigh. Any one of those issues would be more than enough for most people to deal with. The fact that you have to worry about all of them is so sadly, monstrously unfair.

Martha said...

Your anxiety is not unfounded, how I wish it were.

kim said...

There is a book written by Amy Kuebelbeck called Waiting for Gabriel. It's a true account of a woman's pregnancy with a little boy with HLHS. You will sob if you read it, I did. But it does have a lot of information about HLHS. Supposedly it has something like a less than 5% chance of recurrence in subsequent pregnancies. I know from reading your story that you haven't had the best of luck with odds but I'm praying that you won't have to worry about this next time.

Anonymous said...

hey i hope you are hanging in there, why they did not do the operation to the babies