Just this week, we had five doctor appointments, two hospital tours, two fetal echocardiograms, and two follow-ups. This post is a little all over the place, but here is what came of everything.
On Monday, we saw Dr. Silver, my perinatalogist for a follow-up visit to measure the baby’s growth. Luca weighed in at 1lb. 3oz., was in the 42nd percentile for size, and, aside from his heart, everything looked great. My HCG levels were a tad higher than they should be which could suggest growth issues. This is not of major concern at this time because my levels were not majorly elevated and they are already monitoring the baby so closely. However, I’m sharing this because it is imperative that HLHS babies grow strong before delivery for them to handle the surgeries, etc., that they face when born.
On Wednesday, we visited Children’s Hospital LA to see what they had to offer. The fact of the matter is that, according to US News + World Report, CHLA is ranked in the top 10 nationally for Pediatric Cardiology and Heart Surgery (CHLA is #7 vs. UCLA, which is #26). We had another echo done and met with the head of their pediatric cardiology program, Dr. Pruetz. Dr. Pruetz found the same issues as Dr. Sklanksy when we were at UCLA last month. Fortunately, this means no new problems, but I was secretly wishing Dr. Pruetz would just find a perfectly healthy heart…
Dr. Pruetz agreed with Dr. Sklansky’s findings of HLHS and the area of most concern was a restricted atrial septum (click here for more info or see the upper right hand tab). In my first post, I mistakenly called this issue pulmonary stenosis. The combination of RAS and HLHS occurs in about 6% – 11% of fetuses/infants. After the birth, oxygenated blood returning to the left atrium has great difficulty crossing into the right side of the heart and out to the body. Thus, blood oxygen levels in these babies are usually very low, and the babies are very sick and unstable immediately after birth. In utero, the difficulty in crossing the right side of the heart backs up pressure into the lungs. The valves end up muscularizing and the pressure damages the lungs, both of which are happening now and are irreversible.
We now have two options. One option is to intervene surgically, in utero, by placing a stent in the atrial septum to hold it open to allow blood to easily flow and not cause pressure to the lungs. However, the in utero surgery is still experimental, very few places in the country offer it (we’d probably have to go to Boston), the risk of not carrying to term is higher (yet again), the baby could outgrow the stent in utero, and it’s not a permanent fix. The biggest benefit is that it’ll only stop any in utero damage, but would not have an effect on the baby once born. Our other option is not to intervene, but be constantly reminded of the fact that irreversible damage is happening now. Either way, the baby will be taken immediately after delivery to open the atrial septum all the way. He’ll either go straight to the Cardiac Catheterization Lab or OR, depending on the course of action. Then, he will need his first of the three surgeries (called the Norwood) within a few days. Time is of the essence is opening the atrial septum and then having the Norwood. However, the baby needs to be big and strong enough, specifically the lungs now in our case, for the surgery. It has been made very clear to us that we are now in the highest risk group of HLHS patients for mortality.
When we saw Dr. Sklansky at UCLA on Thursday, he was looking to see if this restriction in the atrial septum was any worse. I don’t think he had an actual measurement from my initial visit, so he could not quantitatively tell us if had gotten smaller. However, he was able to look at it and determine that it’s extremely concerning because they can measure the the amount of pressure going back into the lungs. He essentially said the same things at Dr. Pruetz at CHLA. Additionally, we met with the surgeon at UCLA, Dr. Reemtsen, and were pleased. He gave us a run down of the procedure, what to expect, etc. A lot of what he said I learned through my own research prior to our meeting. We also toured L&D, the CICU and NICU, and met with the neonatologist. It was a very long day, but we got a lot of questions answered.
I emailed Dr. Pruetz some follow-up questions after our appointment and below is part of his response. I am sharing this to give a clear idea of what we’re being told to expect. We are praying that God will miraculously heal baby Luca and that the doctors would be wrong, but we also understand the realistic prediction given the science.
However, as we discussed that is not the whole story with HLHS and restrictive atrial septum (RAS) as there can be damage to the pulmonary vascular bed in utero that may irreversible. Thus, the risks for surviving the Norwood procedure and beyond, even after successfully opening the atrial septum after birth, are still not great. To put it another way, patients with HLHS and RAS are still in the highest possible risk category regardless of opening the septum after birth. There is not a lot of data published to support a significant difference if the atrial septum is opened in the first hour or second hour if there is restriction but still some degree of shunting that can occur. It does likely make a big difference if the atrial septum is intact and no blood can cross. However, letting the infant go for many hours untreated with a restrictive atrial septum is not a good idea and could lead to significant risk for injury and death. The next question you should consider is if you are interested in pursuing a fetal cardiac intervention to open the atrial septum in utero and potentially prevent further injury to the lung vascular bed during the remainder of the pregnancy and also to alleviate some of the atrial restriction that would be present at birth.
Prior to my appointments at CHLA and UCLA, I searched high and low for a direct comparison of each hospital and pediatric cardiology program. If anyone is following our journey and is in the same boat as us, I will post about my experiences at both so far and maybe it’ll help. Both programs are renowned and if both weren’t right in my area, I wouldn’t question care at either of them. This is a good problem to have, right?
So, what now? We need to make a decision to continue care at UCLA or switch to CHLA. We need to determine whether or not to pursue the in utero surgery. And, we need a lot of prayer. Below is what you can specifically pray for if you think of it.
Please pray that…
- Luca would continue to grow healthy and strong and would be delivered full term, weighing at least 6.5+ lbs.
- There hasn’t been damage done to Luca’s lungs already and that somehow the pressure that the doctors are seeing will not cause damage
- We will make the right choice regarding the in utero surgery
- We will make the right choice in care at either CHLA or UCLA
- Our work schedules would continue to allow us to take these all day appointments and would coordinate in our favor based on AJ’s travel schedule