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.


Showing posts with label Heart Defect. Show all posts
Showing posts with label Heart Defect. Show all posts

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.

Tuesday, February 10, 2009

November 10, 2008

There are two dates every month that make me catch my breath: the 10th and the 12th. November 12th was the day our babies were born still. But November 10th was the day it all started falling apart. The 10th is the day that I clearly remember, that I replay in my head over and over. . . after that, it’s all a bit of a blur. Today marks three months from the 10th.

I know this post will be lengthy. Feel free to skip it (but come back tomorrow!) But the memories are so clear in my head, I have to write them out. . .

November 10 was a Monday. We had spent all weekend cleaning out the office-to-be-nursery. B put the cribs together. We stood back and looked at the room and the two cribs together and just said: “Wow! We’re having twins!”

FROM BAD
Monday morning I went to court to argue a motion. I was wearing my favorite black pantssuit and red heels. And I won. That afternoon we went for our “big” ultrasound at our MFM’s office. They looked at and measured every perfect inch of our tiny babies. For over an hour B and I watched them and talked to them, and fell more in love with them.

Baby A looked perfect. But while looking at Baby B, the tech said she couldn’t get a good picture of her heart. She called in one of the MFM doctors, who agreed that she couldn’t get a good picture, but seemed to think there might be a problem with the left side of her heart. She scheduled us to come back in one week and retest; hopefully it would be easier to get information at that point. Baby B was smaller; maybe it was just too early to see well.

The doctor also asked for permission to rerun our amnio test for Baby B. She thought that Baby B’s heart problem (if there was one) could be a sign of the generic condition DiGeorge’s Syndrome – a condition illustrated by heart defects and small stature. While she still hoped that everything was fine with Baby B, we discussed that, if the next ultrasound did not show anything different, we would have to meet with a pediatric cardiologist. Baby B would likely need surgery immediately after delivery.

We went from the ultrasound to my OB’s office, where we continued to discuss the ultrasound. After about 30 minutes, my OB switched topics and said that I was spilling protein in my urine. "Do you have an infection?" she asked? "Anything going on?" "No, no" I said, "Nothing that I haven't already told you." She wanted to do bloodwork "stat." We said okay and didn’t think much of it. B and I didn’t know anything about protein, except that I was supposed to eat it! As we were leaving I did stop to ask her – was any of this related? “Humm, good question,” she said, almost thoughtfully. “I don’t think so.”

It was near 5:00 by the time we left my OB’s office. I remember calling my mom as I pulled out of the parking lot: “Mamma. Try to not freak out. And I don’t have any answers. But they think something’s wrong with my little girl.” And as I was saying those words, I saw the lab car pulling in the parking lot to pick up my blood samples.

TO WORSE
I went home and changed. Black yoga pants and a yellow hoodie now. And we started making supper – steak fajaitas. Around 7:00, we were about to fill our plates when my OB called.

She said that my liver enzyme levels were up and my platelet count was down. She wanted me to go to the hospital that night so they could rerun the tests and monitor me for “a few days” – or at least long enough to do a 24 hour urine test. This was all gibberish to me – I’d never heard of enzymes or platelets before – but I knew she was looking out for me and my babies so I went along with it. She said to just go triage and they would decide where to put me. She had a c-section at the hospital that night, and she would check in with me once she was done.

I tried to eat our dinner and help B clean up the dishes. But I did understood enough to be flustered. So he cleaned up while I packed my bag. I packed my makeup, my straight iron, some comfy clothes, a book. . . B said he would go to work for me the next day and pick up some files. As far as I was concerned, I was checking in for a few days of rest and relaxation.

As I was packing my bags my mind necessarily went to the “worst case” scenario. The worst case I came up with would be bed rest – perhaps even hospital bed rest – for the remainder of my pregnancy. That was a looong time, and the thought was terrifying to me. I would go crazy. We’d go broke. If I had to stay in the hospital, how would I get to see enough of B? We spend all our time together. And what about the holidays? . . . . But, I bargained, I would absolutely do any of it, with a willing and happy spirit, if Baby B would just be okay. I knew one thing probably had nothing to do with the other, but bargaining’s never rational. Besides, I was focused on our babies. Particularly Baby B. Potential bedrest – well, that would be my burden to bear. Just so long as the babies were okay . . .

We got to the hospital around 8:00. At some point my OB must have mentioned preeclampsia or toxemia to me, because the nurse at the desk kept asking me why I was there (even though she was supposedly told to expect me), and I didn’t know anything to say other than “suspected toxemia or preeclampsia.” She didn’t like that answer; she wanted an actual diagnosis to put into the computer. I told her I didn’t have one.

We waited and waited, and were finally admitted and in our room sometime after 9:00. I joked with the nurse about how I was finally going to get to catch up on my leisure reading. I'd been resisting reading T.wilight, but this seemed as good a time as any to start.

We were in a Labor and Delivery room, although I didn’t think anything of that, and the first nurse came in around 9:30 or 10:00 to put in an IV port. She said it was just so they didn’t have to keep sticking me; they could use the IV port for everything. Imagine my surprise when the tech came to take my blood and she stuck me again! All I knew at this point was that they were going to rerun the tests that were conducted at my OB’s office to make sure the results were right.

Sometime after 11:00 I convinced B to go home. He had to work the next day, of course, and I preferred that he be at home with the kids (our four-legged ones) anyways. He made me promise to call him if anything happened, no matter what the time, kissed me and went on his way.

TO - there's no words
Not more than a few minutes later my OB came in. I had just been digging in my bag for my book and was settling down to read. She asked where B was. I said that I had sent him home: “Why, do you need him?” She said: “Yeah, I do. Can you ask him to come back?” So, I called B. He didn’t ask any questions, but just got in the car and was back in minutes.

My OB came back in. The nurse was with her, as was someone she introduced as Dr. C. with MFM. That should have told me something right then: it was midnight, and one of the best doctors in Na.shville was in my room in a mock turtleneck. Things were bad.

Dr. C. said that I had HELLP Syndrome, which was very serious, and the only way to “cure” it was to deliver the babies and placenta. I looked him calmly in the face, gave a half-laugh and said, “Well that’s not good!” I thought he meant we’d have to deliver early and they’d be premature.

He kept telling us how serious the disease was, and finally they said that they would leave us alone and give us some time. That confused me. When they left, I finally looked at B and it hit me – they want to take our babies now! Oh my gosh, that couldn’t be possible – it didn’t make any sense at all! The doctors came back in and I started arguing. Actually, those who know me would expect me to argue. But it was more like I was pleading, grasping at straws.

I was just 19w2d at this point. I asked them to please just keep me here in the hospital for a few weeks, I promised I’d be a good patient! Dr. C. explained that my liver and kidneys were shutting down. He tried to be gentle. He tried to be soft with his words. But we weren't getting it. I just kept shaking my head back and forth. I would do what it took to save these babies. I remember thinking, "Hey! You say my liver's not working - but isn't that what dialysis is for?!" There has got to be something we can do!

Finally Dr. C. said: “You’re dying. Your organs are shutting down. You will not live five weeks." In other words - "You will not live long enough to give your babies a chance.”

It didn’t make sense, I had just happened to have an appointment that day. There had been no signs or symptoms! B and I weren’t buying it. “What would have happened if we hadn’t happened to go to the OB today?” B asked. Dr. C. responded: “Within 48 hours she would have seized or hemorrhaged.” When it strikes early, it progresses quickly. He said: “There is nothing you can do.”

Moving on to practicalities, I then tried to get them to let me go to work the next day and get my files in order. “I’ll come back tomorrow!” I promised. They just shook their heads at me. Then I tried to convince them to do a c-section. The thought of having to deliver terrified me. Labor had always terrified me anways, and I could not experience that, go through that, knowing what would be the result. But a c section was dangerous considering my propensity to bleed and hemorrhage, and it could potentially scar my uterus.

They left us alone again. It was probably 1:00 in the morning by now. And I just – I don’t know what happened. I didn’t freak out, I don’t think. I remember crying, but I wasn’t hysterical. I was just so tired. I just wanted to curl up with B. They kept asking us if they could call anyone. “No, no.” we said. “Its just us.” Why would anyone want to come in town for this? But I did call my mom (who of course promptly collected my sister and began the 3 hour drive). All I said was: “Mamma, I’m sick. And they want to induce me. I've gotta go." After my call earlier that day, she must have been so confused.

Finally the doctors came back in. They must have been able to tell we’d finally been defeated. I climbed back in bed and got under the covers. And they began the magnesium drip to try to prevent a seizure. They put me in compression stockings to prevent a blood clot. And they gave me drugs to induce labor. And the wait began.

My blood pressure was spiking dangerously. There were issues with my pulse and oxygen levels. I wasn’t allowed any food or liquids. The magnesium made me weak and I wasn’t allowed out of bed. My red blood cell count was dropping and I was severely anemic. They were worried about a pulmonary embolism, and brought in an x-ray machine to look at my lungs. Whatever was going on with my kidneys and liver. And something about my heart and calcium levels? Who knows. . .

The doctors didn’t see any chance of an epidural because my platelets were so low. No one had told me this. They offered me demerol for the pain, but it made me nauseous. Finally, with lots of steroids, my platelets rose enough that the anesthesiologist agreed to an epidural.

And B just sat by my side and kept hitting that epi button. Just kept trying to make it all go away.