Here’s my reaction from yesterday. I’m assuming you already read Dave’s write-up. Hopefully, by getting it all down and out of my head, I’ll be able to relax a bit more. I don’t think I took yesterday as hard as Dave because I did my grieving after the first visit, but my waking up at 5:30 this morning says that I’m still deeply affected.

Yesterday was long. Over 5 hours of appointments, we talked to 9 different people: a NICU neonatologist, a social worker, a sonographer, a pediatric cardiologist, a perinatal physician, a research study representative, another social worker, a pediatric cardiac surgeon, and a cardiovascular child life specialist. Additionally, we toured both the NICU and the Cardiovascular Care Center (CVCC, the cardiac specific ICU at Children’s) .

Yesterday was a mixed bag. On one hand, it feels good to have a definite plan. On the other hand, medically it felt like we took two steps back, and the plan is pretty crappy. I’d use a different word, but with a kid coming, I need to cut back on my swearing.

The Bad

The echo was consistent with the previous times, with no relative improvement. She is still borderline HLHS. They still won’t be able to decide what surgery she needs until after she is born. It was decided that our daughter’s heart is so bad that we are skipping the NICU, and going straight to the CVCC. One drawback to that is the CVCC is not as used to babies. That makes it less likely that we will be able to hold her while she is there. Additionally, there are no webcams at the CVCC like at the NICU. That will make it harder for Dave to feel connected when he has to work. Of course, we are already working on a plan to provide our own webcam using our baby monitor.

It was also decided that PGE needs to be started in the first hour. That means she has to be immediately hooked up to all the bells and whistles of a hospital. The after birth time will be nothing like normal, and has the potential for great sadness. At least the cardiologist took the time to explain exactly why we are doing this, and I have to agree with the decision.

Another implication of her unknown heart status at birth is that she will not be receiving any nourishment by mouth for the first bit. Since they don’t know how effective her blood flow will be, they don’t want to divert any away from important things like the brain to go to the gut for digestion. She will get all her nutrients from an IV. Once she does start eating, it will be a slow ramp up that takes at least a week. I guess I’ll become well acquainted with pumping.

I was also sad that the set of 3 surgeries to replumb an HLHS heart were so strongly back on the table. I was so hoping that it would just be a simple aortic repair. It still might be, but it is not as hopeful as after echo #2. At least the surgeon took the time to explain exactly how they will be deciding if our daughter ends up with 1 or 2 ventricles. I am happy that it is extremely data driven, with a few separate statistical models for decision making in the 3 separate calculators they will use.

If we do have the set of 3 surgeries, our summer will be very different than we had hoped and planned. Yes, we were leaving 2 months unplanned, but we had started putting trips on the calendar for when she was 3 months old and beyond. Now, we might not be able to do those trips. Even my brother’s wedding in September in Boston has the strong likelihood of being impacted.

No matter what, she needs surgery before she comes home, at least to expand the aorta. That surgery can’t happen until she’s almost a week old. That puts minimum time at the hospital to over 2.5 weeks. A month is more likely, and 2 months is not unheard of.

We still don’t have buy in from the hospital staff about our ideal birth scenario. Talking with the NICU staff made me realize that we need strong buy in to keep anything resembling normalcy in our birth experience. I wrote a long email, and am waiting for a response. At least I’ve been warned. NICU people are very intense.

Another crappy thing is that we are still fighting billing for our first visit in December. I hate how, just because Allina and UCare are having a pissing contest, we pay the literal price in terms of thousands of dollars.

The Good

With the two tours, we have a good idea of what to expect for the time we are at the hospital. For a hospital, they try really hard to make it a pleasant experience with lots of resources, like a parents’ area in every room, room fridges, the Ronald McDonald house, kid friendly accessories, etc.

The Mayo Clinic is running a research study for HLHS babies that involves cord blood. Dave and I had it on our list to look into it. Instead, a study rep came to us and invited us to join the study. They are just entering phase 2, which means phase 1 showed safety and improvement. The surgeon was even excited about it.

Even though the aorta has crappy flow, the heart has good function. It is unknown if the left ventricle has adequate function, but it looks good. After birth, they will do two different echos, one immediately in the first hours, and then another at around 3 days. There, they can measure the specifics like left ventricle ejection fraction, etc. Those numbers will be fed into the calculators to decide if the left ventricle is adequate. It won’t just be one doctor’s gut decision.

She is still growing normally. She is at the 67th percentile, with an estimated weight of 4lbs 9oz. Of course with Dave’s big head, her head hit the 90th percentile. It was cute to see hair on the ultrasound. It might even have a bit of a curl.

We are happy with our decision to be at Children’s Minnesota. They handle more cases like this than both Mayo Clinic and the University of Minnesota combined. They do less than Boston, but the advantages of a local hospital win out.

I am relieved that they are okay with us staying with the midwives from the Minnesota Birth Center. The normal procedure for congenital heart defects is to have labor induced. I don’t want that. With the midwives, that is way less likely, and I am pretty confident they will only induce if medically necessary. Additionally, the chances of an uncomplicated natural vaginal birth are much higher with the midwives. That will make it more likely that I can go with our daughter to the CVCC, and we won’t be immediately split up.

I think that is it. They decided that will be our last echo before birth. Hopefully, I won’t see any of those people until after a peaceful, natural birth in April. Now, let’s see if I can actually get some sleep.

Today we had ultrasound number three with the Perinatal Physicians. Audrey is at 32 weeks tomorrow. We got a 3rd chance to see how our little one’s growth has progressed and talk in detail for the plans for birth.

The short version is, not much change. Our daughter will be in the Cardiac Intensive Care Unit within 30-60 minutes of being born. We were hoping for a little more bonding time before that happened but everyone believes it is critical to get on PGE (the drug that stops the ductus in the heart from closing) as soon as possible. They will also be doing the heart echo and other tests then determining what is needed. It’s all but certain that some kind of heart surgery will be needed within the first 1-2 weeks. If she has full-blown HLHS, it will be the scary first of three surgeries to re-plumb her heart into a 2-chamber vessel. The better outcome will be she only needs a surgery to widen her aorta.

It was an emotional day. It’s getting more and more real that we’re having a baby and the realization of what the heart issues mean is growing. There’s going to be ventilators and monitors and IVs and no telling how much, if any, holding of her we’ll be able to do.

It’s scary not knowing what will happen. If she has full-fledged HLHS, it’s a very tough and challenging process. The period in between the first surgery, immediately after birth, and the 2nd surgery, around 4-6 months, is a fragile period where we have to watch out for illness and monitor things very carefully. It’s amazing to think that we are hoping she’ll only need an aortic graft!

This was the last big ultrasound with the fetal echo before birth, unless something else comes up. At birth, there will be a team from the neonatal intensive care unit to do the immediate assessment. Hopefully we’ll get a little time with her before they whisk her away to get started on her journey.

I am finding this tough. I’m a fixer. I want to do something to help fix the problem and all I can do right now is prep and be supportive to Audrey. At birth, I’m going to initially have to split my time between Audrey and the baby as the little one will likely be at Children’s before Audrey is stable and able to make it over to us. Big breath. Lots of emotions.

Even with the above, I am still extremely happy we have resources like Children’s Hospital so close by! It was helpful to meet all the staff, from the cardiologist to the surgeon, the perinatologist, care coordinators and social worker.

Thanks to all the friends and family who are helping us in this process. We appreciate the support and we’re going to keep needing it.

Yesterday, we had our second appointment with the heart doctors at Children’s Hospital. In brief, our daughter’s heart is still too small on the left side, but it did look better than the first appointment six weeks ago. Her aorta especially looked better. Her overall growth is good, 78% percentile. Of course, per Dave’s big head, her head is in the mid 80’s percentile-wise. We have another appointment, and possibly last prenatal appointment, in four weeks. Ultimately, nothing will be known or decided for certain until she is born.

It was nice that this appointment was only four hours instead of last time’s six hours. It was still full of all sorts of people, from the ultrasound tech, to the perinatal physician, pediatric cardiologist, social worker, and clinical care coordinator. Next time, we also get to meet with the NICU and the cardiac surgeons. Those are the people who have the most say over our daughter’s care.

The pediatric cardiologist was the same one as last time. Her reaction was extremely encouraging about how much improvement she had seen. The mitral valve is still probably the worst part of the heart. She gave us a new possible diagnosis to look up: Shone’s Syndrome.

Children’s Hospital has not received anything from Boston. We gave them the verbal report that Boston gave us, and Children’s agreed that she could have a two ventricle heart. They even agreed that the possibility existed that she might not even need the scary side effects drug PGE, but they cautioned that they like to be conservative, start the drug, and then stop it if not necessary.

Dave and I came with a whole page length of typed questions. Many of the questions involved the plan from here on out. They took the time to answer all our questions. It was very encouraging. In general, I am very happy that they had no problem with us staying with the midwives from the Minnesota Birth Center for both the weekly follow-ups starting at 32 weeks, and for delivery. We still have a few points of contention, but the conversation has been started. They have been warned what I want should medical conditions allow, and that I can be stubborn.

Dave will share his thoughts a bit later.

About a week ago, I contacted Boston Children’s Hospital for a second opinion. They are the world leader in hypoplastic left heart syndrome (HLHS), and do many things that other places don’t, such as fetal surgery. 

Monday, they finally received all of our medical records. A doctor, Dr. Friedman, reviewed the results and actually had better news.

By the way, this is a long post. If you don’t feel like reading it all, skip down to “In Summary,” near the end. 

Boston does not believe that the diagnosis of HLHS applies. Yes, they see that the left side is smaller, but Dr. Friedman thinks that it will very likely be big enough to pump blood to the body. He does not think that the series of 3 surgeries will be needed.

They also did not see any aortic stenosis. That means that no fetal intervention is recommended. So, I will not be going to Boston for fetal surgery.

The diagnosis that Boston believes is most accurate is Coarctation of the Aorta (COA), or a narrowing of the aorta. This is, in general, less serious than HLHS. It will still require repair, but is much simpler than the series of 3 surgeries for HLHS. Plus, COA repair has a much higher survival rate than the HLHS 3 surgeries.

Boston believes that our local Children’s Hospital should be able to handle this. Here is the information page about COA from Children’s Minnesota. Boston wants us to mention this diagnosis to our local care team and see what they have to say. Boston reconfirmed that I need to deliver our daughter where we are planning on having her surgery. Right now, they see no reason why we should have to go to Boston. However, they would like us to call them back after our next follow-up fetal echo cardiogram on January 17th.

In Summary:

In short, we received good news this week. Boston believes it likely that our daughter will have two pumping chambers. Instead of the diagnosis of HLHS, they think coarctation of the aorta (COA), or narrowing of the aorta, is more applicable. No fetal intervention or surgery is recommended at this time. In fact, they do not see the need for us to go to Boston at all. However, we are still planning on a heart repair once she is born, with extended NICU time.

We both feel some relief, and slept better last night. Our daughter is not out of the woods. We still have a long, hard journey ahead of us. But at least there is a bit more hope. We still welcome thoughts and prayers.

Please read Dave’s Journal Entry first. This entry assumes that you have already read his.

Thanks for coming to our website. If you know me, you probably know that I don’t share personal things easily. This situation is no exception. Please know that I welcome any questions you have, at any time. Even if I do not offer the information, I am willing to answer questions. Don’t hesitate to ask. Dave and I are slowly mastering the skill of talking about this without tearing up, and they say practice makes perfect.

I attached a picture of a normal heart vs. a hypoplastic left heart syndrome heart. It is from the CDC article Dave linked to. Our daughter’s heart is not quite as bad as the one in the image, but it gives the general idea.

Right now, we are waiting to hear back from some questions we asked on Monday. There is a small chance of fetal surgery, possibly at Boston Children’s Hospital. We should know more in a week. Otherwise, it is just increased monitoring of this pregnancy, and waiting until she is born.

That moment, minutes after she is born, that they take her away will certainly be a sad one. We welcome any thoughts and prayers.

It’s been a wild week. Last Thursday Audrey and I learned that our little one likely has a Congenital Heart Defect. Specifically, she has an issue called Hypoplastic Left Heart Syndrom, or HLHS. HLHS is a situation where the left side of the heart is underdeveloped and can’t function properly. There is no danger right now while our little one is inside but when she starts to breathe on her own, if untreated it would likely be fatal in several days. 15-20 years ago, it would be fatal. Luckily, there are some risky, but apparently effective procedures that can restore a mostly normal way of life.

So what happened? Audrey’s now into her 22nd week of the pregnancy. At the 20 week Ultrasound, the Dr from the awesome Minnesota Birth Center thought he detected a 2-vessel umbilical cord and possibly a smaller left ventricle in the heart. While the Dr was formerly a Perinatal Physician, he hadn’t been in that field for a few years and recommended a second look. Audrey and I had to wait 1.5 weeks but we got in to see the staff at the Minnesota Perinatal Physicians at Children’s Hospital in Minneapolis. After a long series of detailed ultrasounds, we talked to a Perinatal Physician, a Neonatal Cardiologist, a Genetic Counselor and a Care Coordinator. They gave us the diagnosis of HLHS. 

Our diagnosis is currently borderline so we’re hopeful. The current, worst case process for dealing with this will involve a series of 3 open heart surgeries. One immediately a few days after birth. Another around 4-6 months and another one somewhere at 18 months to 5 years. The procedures basically convert the heart from a 4-chamber organ to a 2-chamber organ.

We’re scared, unsure and sad, but also extremely happy to know about this now, before our little one is born rather than learning at birth and having to deal with all of this all at once. 

We’ll share more soon but wanted to share some basics. 

If you want to learn more, here’s a link to an article from the CDC with some Facts about HLHS to start you down the rabbit hole.

In short, Audrey is healthy and safe. Little baby Royer is healthy and safe while inside mom. This doesn’t affect the way we handle the pregnancy right now other than additional monitoring.

Our 20 week ultrasound was not a happy day full of pretty pictures, but instead the start of a long journey to address a critical congenital heart defect. Follow along by reading our Journal.

These journal entries make the most sense if you read them all, from oldest to newest. If you haven’t already, we encourage you to read from the first entry, “A Turn of Events.”