5/31/18 UPDATES!!

Yesterday my mom, Brian, and I were in Houston at The Fetal Center at Children’s Memorial Hermann. We had the following schedule:

1. 8:30 a.m.–Echocardiogram with Dr. Gardiner. Our normal cardiologist, Dr. V., is on vacation. Apparently doctors vacation too! Who knew 🙂

2. 10:30 a.m.– Growth Ultrasound with Dr. M…Our normal fetal specialist, Dr. Johnson, is out of the country at the moment.

3. 11:30 a.m.–NICU tour

4. 12:00-12:45– Tour the RV park where we will be staying

5. 1 p.m.–Meet with a neonatologist who will be taking care of Daniel after birth and after surgery

6. 2:30 p.m.–OB-GYN appointment with the doctor who will be delivering Daniel

It was a full, jam-packed, exhausting day. We left our house at 3:30 a.m., arrived at The Fetal Center just in time for the echo, left Houston at 6 p.m. that evening, and arrived home around 10 p.m.

When we were there last time on April 4, I found out a lot of information about what HLHS is and about the treatment plan. I had no experience with HLHS and didn’t really know anyone who had it, so I had no idea what questions to ask. I didn’t have a clue about the complications that can occur, about how many things can go wrong, and about what we were up against with this diagnosis.

This time, I was prepared. As I have described in previous posts, I now know people now who have this and who have children with this. I am very familiar with the plethora of complications that can arise, seemingly out of nowhere. Over the last 2 months as the overwhelming truth has set it, I have felt disheartened and scared.

Here is a breakdown about how a normal heart works: 

1. Blood that needs oxygen enters the heart from the upper body (through the superior vena cava, SVC) and from the lower body (through the inferior vena cava, IVC).

2. The unoxygenated blood goes from the SVC and IVC into the right atrium.

3. Then it moves through the tricuspid valve into the right ventricle.

4. The right ventricle pumps the blood through the pulmonary artery into the lungs.

5. The lungs give oxygen to the blood.

6. The newly oxygenated blood then travels back into the heart through the four pulmonary veins. There are two pulmonary veins on the left side and two on the right side, all leading into the left atrium.

7. The blood goes into the left atrium and through the mitral valve, into the left ventricle.

8. The left ventricle, the strongest muscle in the heart, pumps the oxygenated blood through the aorta to the rest of the body.

Daniel has a textbook case of HLHS. His mitral valve is completely closed, his aorta is very small and closed, and his left ventricle is severely under-formed and doesn’t function. This means that Daniel’s oxygenated blood (from the placenta in utero) isn’t going to his body the normal way.

In babies in the womb, there are two holes open in the heart. Daniel’s oxygenated blood is leaking through these holes into the top part of the aorta, which then carries it to the rest of the body. Shortly after birth, these holes normally close. If these holes close, Daniel will die. There will be no way for his blood to get to his body.

Immediately after birth, Daniel will be started on IV prostaglandins, which should keep the holes open. Yesterday, the doctors were looking at his heart and trying to see the following things:

1. Is the atrial septum (one of the holes in his heart between the right and left atrium) still open? If this were to start to close, then it could seriously affect his pulmonary veins. Remember, there are 4 pulmonary veins that bring oxygenated blood back to the heart where it is then pumped to the body. If those pulmonary veins start to close, it is called pulmonary vein stenosis, and that is very bad. At the moment, Daniel’s atrial septum is open. We have to have another echo and ultrasound in a month to be sure it remains that way.

2. Is the tricuspid valve functioning properly? They were looking to be sure it is not leaking or malfunctioning somehow. Daniel’s tricuspid valve looks great.

3. Is Daniel’s right ventricle pumping well? It is.

Apparently Daniel has textbook HLHS, which is AMAZING news. The fetal cardiologist, Dr. Gardiner, broke all of the above information down to us. She was amazingly helpful!

I can’t stress this enough, y’all. I can’t believe how happy I am to have received that news yesterday. Two months ago, that was the most devastating news I had ever heard. Now, after seeing true pain and suffering in others, after having my eyes opened to the vast possibilities of heart defects, I am so incredibly thankful we are not facing anything worse. Not yet, anyway.

After our echo, I had another ultrasound, which monitors Daniel’s growth in the rest of his body. Thankfully, Daniel looks great! He is right on target, and his other organ systems look good.

I felt giddy, like I was on cloud 9. I didn’t realize I had been *metaphorically* holding my breath for weeks. After our echo and ultrasound, I let out a big sigh of relief.

We then took a tour of the NICU. I felt like an old hand while following the nurse around. Hannah’s experience in the NICU means that I am familiar with the rounds, with the machines, and with life there. Doctors and nurses make rounds in the morning at 9 a.m. There are multiple “pods,” or rooms, where babies are held, each pod named after a different animal. Because Daniel is a cardio baby, he will be in bear pod. They showed me the pod and the different kinds of machines babies are held in. They also showed me the breast pumping room and the overnight rooms. At Children’s Memorial Hermann, there are multiple rooms where parents can sleep overnight or take naps right in the NICU.

Immediately after birth, Daniel will go to the NICU. He will be given an IV and another full body workup (echo, etc). His entrance into the world will a bit more traumatic than usual. The neonatologist explained it this way– there are only so many things that ultrasounds can detect. She said it is like a tree in winter. They can see the trunk and the branches, but the edges of the tree and the individual limbs are unknown. After birth, they are able to see much more, which is why they have to do so many tests.

The neonatologist told us that every baby is different. If everything goes well, Daniel will look and act just like a normal baby after birth. He will go to the NICU for 7 days where he will practice sucking and eating and recuperate after birth. A week after birth, he will have his first open heart surgery.

After surgery, Daniel will probably have an open chest. Over the years, doctors have learned that it is best to leave the chest open because of swelling. After a couple of days, Dr. Salazar will close Daniel’s chest.

Also after surgery, Daniel will be intubated (on a breathing machine). Hopefully, doctors will be able to remove this shortly after surgery, but it will probably stay on for at least a couple of days. This looks like a big mask with a tube sticking down his throat.

Daniel will also have internal leads. If you have ever had heart leads stuck to your chest, you kind of know what this means. Instead of being attached to the outside of the chest, the leads will be attached to the INSIDE of his chest. So he will have multiple wires sticking out of his skin, with leads on the inside monitoring heart activity to be sure his heart settles into a normal rhythm after being on the bypass machine.

He will also have a catheter, or multiple catheters, sticking out of him. This is so doctors can give him medicines directly into his heart, apparently (or something).

Dr. Salazar says Daniel will take about 7 days (at best) to recover from surgery. This will mean at least 7 days of not holding Daniel and seeing him like this.

If I thought I was on cloud 9, after this conversation with the neonatologist, I was giddy no longer. Surgery is always risky, and open heart surgery on an infant is even more so. Even with the best case scenario, there are so many things that can happen.

The neonatologist, Dr. Lopez, says that every baby is different. Once babies are born, their lungs start to actually breath oxygen. In the womb, the lungs aren’t actually working because the placenta gives oxygenated blood to the baby. So, after birth, many things can happen once the lungs and heart actually start working on their own. That is when many scary complications can happen. With HLHS babies, there is not as much oxygenated blood getting to the body compared with normal babies. The first place oxygenated blood goes is the brain, and one of the last places is the bowels. A very scary complication that can arise in the 7 days between birth and surgery is necrotizing enterocolitis (NEC), among other things. Doctors will be heavily monitoring Daniel for this immediately, so if he develops something scary, they can intervene quickly. Between birth and surgery, Daniel will practice sucking, but doctors will be heavily monitoring how much milk he receives so that they can be sure his bowels aren’t overloaded, which can cause NEC and other things.

We are praying that Daniel’s lungs and heart function properly after birth. We are praying that his pulmonary veins stay open and that his atrial septum stays open. We are praying for Dr. Salazar and our whole team of doctors. We are praying that he doesn’t develop NEC and other complications between birth and surgery, that surgery goes according to plan, that he his able to be extubated in a timely way, and that he meets no further complications after surgery. I am also praying for the strength to deal with all of this and for trust in God, regardless of what happens.

During lunch, we went to see the RV park where we will be staying. It is only about 10 minutes from the hospital, and it is gated. Our site is under a huge tree, so there will be plenty of shade, and it is right by a grassy area where the kids can play. It is only about 10-15 minutes from John and Christi’s house, too, and there are numerous playgrounds and splash pads nearby. Seeing the place we will stay gave me enormous peace of mind. I am starting to see that we will make it through this.

I also had an appointment with my OB-GYN. I liked her immediately. She is an older woman with this epically long hair–down to her ankles! (side note– I immediately noticed her hair also because Hannah is obsessed with hair, and I laughed imagining Hannah’s face if she saw it). My pregnancy is progressing normally. No worries so far. I failed my 1 hour glucose test (I always fail the one hour) on Tuesday, so I have the 3 hour glucose test this upcoming Monday. Hopefully I don’t have gestational diabetes! My blood pressures are looking great, so I don’t have to worry about preeclampsia yet. Hopefully I stay healthy, and hopefully Daniel stays in as long as possible.  I will stop the progesterone shots at 36 weeks, and I will be induced at 39 weeks if Daniel hasn’t come already.

Thank you all for your prayers. We so appreciate all of our friends and family who have supported us through this journey.