Week in Review

For all you big picture types.

Our daughter Sophie was born six weeks early with two major heart defects. The first was a restriction in the aorta that prevents oxygen rich blood from getting to the lower body. The second is a hole between the lower chambers of the heart that forces the lungs & heart to do alot of extra work. Sophie had a successful surgery to correct the first problem on Monday, January 21.

Recovery from the surgery was a little scary, but alot of that was because we didn't really understand what to expect. She was very pale & puffy on Tuesday, but her looks returned to normal by Wednesday. She was on a ventilator to help with breathing until Thursday. We know now that all of this is common, especially for preemies.

Sophie is now back in the NICU. She is breathing on her own & taking regular feedings of milk through a tube. We'll hopefully have a better understanding of her future treatment after the big doctor's pow-wow tomorrow morning. We tend to get a little wrapped up in what's still to be done, but let's celebrate what an amazing week it's been. In a way, she's in the same place she was last weekend except that one major obstacle to normal circulation is behind us. Awesome!

A Zippy Delivery

10am At the Maternal Observation ward, they hooked Stephanie up to the same fetal monitors as at the doctor's. Her blood pressure was a little high, but the headache had gone and the graphs were looking good. They monitored her for almost 2 hours. We felt like they were about to let us go home. But the OB had called to order a blood test so they came and drew some. Our nurse came in with the results and said "There's a 99% chance you're going to have to deliver today." They diagnosed her with HELLPs syndrome, which best I can tell is an extreme form of preclampsyia with a combination of low platelets, high liver enzymes, and some other stuff.In a nutshell, it can be life-threatening to both mother and baby, and the only remedy for it is to deliver. Stephanie's platelet count put her in the moderately severe category. The nurse said usually they see other symptoms like blurred vision, swelling in the legs, and really high blood pressure. Stephanie had shown none of them except the one high pressure reading in the morning.

(In reading about HELLPs, 65% of patients exhibit a "band of pain" around the upper abdomen, which sounds exactly like what Stephanie had during the latter part of her pregnancy. Also, preclampsyia can lead to smaller babies, since the higher blood pressure means less blood and nutrients get to the uterus. I'm disappointed these two factors didn't lead her perinatal doctor to diagnose this earlier. But I also discovered that HELLPs is a very rare syndrome, occurring in around 1/2% of all pregnancies. Also, as I said above, the only thing they could have done would be to deliver the baby, and then she might have been in worse shape if she hadn't gotten those extra few days or weeks to develop. So while I'm obviously not happy about what happened, I'm thankful that they are both okay and nothing worse happened.)

So we sat there and waited for them to come back. We let what she had just told us sink in. I realized that I had neither my camera, Stephanie's cell phone (I had been planning to stop at tmobile and buy a prepaid phone on the way home from the dr visit that morning.), my parents' work phone numbers, change of clothing, someone to watch the dogs, etc. But things happened so fast I didn't have time to even use the phone til after they took her into delivery.

1pm They decided who would be delivering and wheeled Stephanie next door to labor and delivery. The c-section was going to be done under general aenesthesia in the interest of time. An bunch of nurses, technicians, aenesthesiologists and doctors started coming in. They were prepping Stephanie, giving her magnesium sulfate to combat the high blood pressure and other problems (this made her feel hot and nauseous), explaining things to her, talking to me while I was trying to listen to what they were telling her, trying and failing to get an IV started in her, asking each other who was doing what, asking where was the doctor, oh wait the on call doctor is not doing it but her OB is coming in, calling for spare blood due to the low platelet count, calling the O.R., asking me which NICU the baby was going to after delivery!

This worried me a little bit. I was getting a little scared by what seemed to me like chaos. I asked, "Who is running this show, anyway?" The charge nurse--a large and stern woman--sat me down and explained each step. She had just come in and I think realized how hectic it seemed to me. The reality is probably that they had just bumped us to the head of the line and paged a lot of people from all over to come cover this immediately. Either way, I felt better after I knew the gameplan. (I learned from a nurse afterward that no one in the O.R. even knew that the baby was breach until the OB saw her.)

2:30 Word came in that the doctor had arrived and the OR was ready. They whisked Stephanie off. Suddenly, I was all alone. The room was strangely silent. I made a quick call to Stephanie's mom. She told me she was on her way. Then, I waited.

2:45 The neonatologist came in to tell me they were stabilizing my little girl and then would transport her next door. I interrupted, "It's a girl?" She said, "Oh, you didn't know? It's a girl!"

3pm They wheeled the baby out into the hall on the way to the children's hospital next door and waved me over. I got to see her for about 10 seconds. But I was amazed that she looked perfectly normal and pink. I had been steeling myself for a premie and possible issues, but she looked very normal, just really small.

3:01pm I started calling people on the phone. My brother, my mom, anyone I could find a number for.

3:10 I met our Obstetrician for the first time. She told me Stephanie was fine and would be in recovery for the next 2 hours or so. She looked at me and asked if I needed anything, maybe some orange juice? I said no, I was fine, but realized I hadn't had anything to eat or drink since 7am. She said, "I'm going to bring you some orange juice," and I was glad.

5:00 They brought Stephanie back down from recovery. I shared the news with her.

8:00 I finally got to go over to the NICU and see my daughter. Stephanie was still in recovery and wouldn't be able to go next door and see her daughter for over 2 days. This was really hard on her. I took lots of pictures to show her, and got a good movie clip of the baby crying, but it was still tough for her to wait that long. It was quite a moment when she was up to sitting in the wheelchair and I got to take her across to see our baby girl.

All day long, people had been asking me what her name was going to be. I kept telling them, well we really weren't expecting to have to do this today. Luckily it was a girl because we had no idea for a boy's name. But we knew if it was a girl what we probably wanted to name her. But I wanted to wait until I could discuss it with Stephanie. We did have a backup, though. Since we didn't find out the sex before, we had given him/her a nickname, so we could stop saying "it" or "he/she" all the time. We just called her Zippy.

A Zippy Pregnancy

(So it occurs to me that with all that's been posted, I never really explained why our baby is in the NICU. I'll explain that and then what complications we experienced during the pregnancy. For the short version to just find out what's wrong with her now, you can just read the next three paragraphs. There'll be a later post about the delivery.)

Yes she's premature and that is an issue, but she also is going to need surgery to correct a restriction in her aorta once she gets bigger. (The doctors want her to be 4.5-5lbs and right now she's just under 3.5lbs.) This sounds scary, but believe it or not, it's a routine procedure with a very high success rate of 95% or higher. Also, I'm convinced we have the best pediatric heart surgeon in FTW, maybe all of DFW and North Texas. His resume is impeccable (10 yrs of thoracic and cardio surgery at Johns Hopkins, then for years he did most all the heart and lung transplants for Emery in Atlanta) and he has done thousands of these. It is not even open heart surgery, they go in under the arm on the side of the abdomen to do the procedure. Recovery is relatively quick, recurrence is minimal, and there are no lasting effects once it's fixed. And what we find so encouraging is that she is stable enough that the doctors are comfortable giving her all this time to get bigger and stronger on her own so that it will be an easier surgery and quicker recovery for her.

This restriction is called coarctation of the aorta and it keeps blood from flowing to her lower body as well as it should. The good news is that it didn't endanger her in the womb because in a fetus there is an extra vessel called a ductus that allows blood to bypass the lungs and go into the aorta to the rest of the body. And her blood was already oxygenated for her. After birth, the ductus normally closes as the lungs are inflated and oxygen is absorbed for the first time. For Sophie, her doctors are giving her a medication to keep the ductus open indefinitely while she gets bigger. Keeping it open works the opposite for her. Since her aorta is restricted, blood goes the other way (path of least resistance) through the ductus into the pulmonary artery to the lungs where it mixes and then goes on to the lower body. At least, I'm pretty sure I've got that right. It really is amazing what they can do nowadays.

These kind of heart defects often occur in groups, and she also has a VSD or ventricular septal defect, which is basically a hole between the chambers of her lower heart. This also sounds scary, but it's not that bad. In fact, when it was first diagnosed in Oct, they said she would need immediate surgery in the first few days to repair this, but throughout the pregnancy it got better and now is not even the main concern. In fact, they are going to fix the aorta and give the heart every chance to heal itself and close over the hole with tissue. After a few months, if it's still there and is a problem, they'll go in and put a patch over it. But even if that's necessary, it's still a routine procedure and she will be much bigger and stronger by then. I know this all sounds scary, but believe me, it could be a lot worse and we consider ourselves very lucky to have her be born to us in the condition that she was in.

More about the pregnancy...

Here's a little background on how we got to where we were on January 8th. During the pregnancy, Stephanie's OB ordered a quad-screen test. Hers showed that she might be at risk for a condition called spina-bifida. This is scary stuff to hear. You never want your doctor to call you directly, trust me. If a nurse or assistant calls you, that's something routine. If your doctor personally calls you... uhoh. Anyway, we were referred to a perinatal (high-risk pregnancy) doctor and also had to meet with a geneticist. The good news was that the new Dr saw no signs of spina-bifida in the sonogram.

At our next meeting, however, he said that he saw something a little different about the heart, but could be nothing, so he referred us to a pediatric cardio specialist. She did an echo-cardiogram. This indicated there the above heart defects, but she thought they would be reparable and while they sound scary, they are fairly common, like 8 in 1000. This was months before delivery, however, so things could improve by then. Okay, we accepted that our baby would likely need some sort of treatment after delivery. But she also said we could not deliver at the hospital near our house (I can literally see it out my kitchen window), but would have to deliver in Dallas so the baby would be at a facility that could provide that kind of care.

Well, Stephanie works in SW Ft Worth. She didn't like the idea that she would have to drive 60 miles across DFW for all these doctor visits. Plus, what if she was at work and went into labor during rush hour? It would be impossible to get there in less than 2 hours. So we told her OB that we would prefer to do this in Ft Worth.

This meant that we would have to get a whole new team of doctors, including OB. The delivery would be at Harris Hospital in downtown FtW and the baby would go next door to Cooks Childrens Hospital, so we would have to get new doctors that practice at that hospital. So we met with a new perinatal, cardio, OB and thoracic doctors over the next few weeks. Oh yeah, and Stephanie also developed gestational diabetes, despite her very healthy pregnancy specific diet. So we got to meet with a nutritionist and nurse practitioner about that.

Things were going well. She had no high blood pressure, swelling, hardly any nausea. But she did have an episode where she felt pain in her upper abdomen. She couldn't really identify it, so we called the ob's office. The nurse practitioner on call told her to take a hot bath and try aceteminophen. This relieved things and we figured it was upset tummy. Over the next week or two she had 2 more episodes like this. One was after eating too many rich foods, so we thought it might also be due to blood sugar issues from the diabetes.

But then they got worse. Over the next week she had two or three more. They always happened in the evening and the only relief was the hot bath. The "attacks" as I call them seemed to get worse and take longer to go away. She told both her OB's office and also the new perinatal doctor. They told us it was probably just normal aches & pains from pregnancy. It's not uncommon for nausea to return in the 3rd trimester, either. They hear a pregnant lady say she has trouble breathing and an upset tummy and don't think much of it--especially for first-time parents, I bet.

I was still worried, but what could we do? Besides the baby was always looking good in the sonogram and had plenty of movement the whole time. So we tried not to worry too much.

Thursday Jan 3 we saw the perinatal doctor again. She said the ultrasound looked good, but the baby was smaller than it should be at this point (3lbs vs 4-5). This was enough for her to tell us that we would have to come in for monitoring twice a week until delivery. We set up an appointment for the following Tuesday. In the meantime, we tried to talk to the baby and tell it to get bigger.

Well, on Monday Stephanie had another attack and it was the worst one yet. She had to have someone drive her home from work. She could hardly breathe and the pain lasted for almost an hour. It was very scary for us both. But finally she started feeling better, and the next morning was the doctor visit so we felt better because we could tell her about it then.

I also forgot to mention that for a while there she also had placenta previa, which did correct itself, and that the baby was breach for the entire term. But in actuality the whole thing was less scary than it probably sounds (except for the last attack). We knew that the baby was moving a lot, which the doctors said was great, and on the sonograms exhibited good breathing motions. They "breathe" the amnio fluid into their lungs to develop them and the chest muscles. Again, we were told this is very good and you don't always see it during the short sonogram sessions. So this was encouraging.

Tuesday's visit was not with the doctor, but a nurse or technician who did the sonogram. The baby's movement was really good. We told her about what happened the night before, but she didn't seem that concerned about it. Then they put Stephanie on a fetal monitor to observe fetal heartbeat and contractions. During this she got a massive headache and her blood pressure was high (for the first time ever). The doctor said that there was something not right about the way that the baby's heartbeat was reacting to the contractions. So she sent us down the street to Harris to the Maternal Observation ward so they could monitor her. Our new OB (who I had yet to meet) was also paged and told to expect us there...

What's this all about

I know there's a lot of people that want to know how our baby is doing. She is great. We don't yet know how long she will be in the NICU, but her neonatologist said that she "could not be happier" with the progress. They are feeding her milk now to get her to start growing and putting on weight. We can go see her or call them for updates anytime around the clock. So that makes not being able to take her home with us a little easier.

Unfortunately, we don't have the time to directly tell these updates to anyone except the Grandmas right now. Also, trying to email everyone is proving more difficult than I thought, so my plan is to post any and all updates, news, and some pictures here continuously as I get them. Basically if I think it's something more than one person would want to hear, I'm just going to post it here. You can bookmark the main link to this page brd.erf.net/sophie and always see the newest posts at the top. If you use a MyYahoo page or Google homepage, you can click the appropriate button on the right side of the page here and get these updates to automatically show up right there on your homepage.

To have posts here show up in your Outlook email program, try following the directions on http://office.microsoft.com/en-us/outlook/HA101595391033.aspx When it asks you what to put in for the feed address, cut & paste this in there:

http://brd.erf.net/sophie/atom.xml

I don't use windows, so I'm not able to test this out, but if there's problems or questions, let me know.

Also, when I get time, I'll try to share more about the things leading up to and including her delivery. But for now, she is staying at Cook's Children's Hospital in their NICU. Stephanie did get to come home yesterday and is doing well. She is tired and sore. The hardest part is going to be getting her to keep still for the next 4-6 weeks and not doing too much.

For now, we're trying to get caught up on sleep, news, emails, phone calls, pictures, etc. And of course, we'll be making daily trips to Ft Worth to see our daughter.