If you’ve been following our story, first of all thank you for the support, love, encouragement you’ve shown. We’ve known about baby G’s condition since 17 weeks and for every day since, we’ve been preparing for the moment we’d have to say a possibly quick hello, and eventual goodbye to him. Week after week, echocardiogram after echocardiogram his poor prognosis and probable poor quality of life was reiterated, often with new and more lethal defects found in his heart anatomy and physiology. Barry and I were able to make final arrangements, we’ve visited funeral homes, we’ve planned out his memorial service and what we wanted it to look and be like. We’ve kept our friends and families informed (probably ad nauseam) and have welcomed all the arms we’ve found tightly wrapped around our family.
About a month ago, we were advised due to new echocardiogram findings suggesting the evolution of G’s condition into a hypo plastic left heart variant, to see a surgeon in Tampa. If you have been following, we saw him and one of his colleagues and after another echo and another new finding of intact atrial septum, we made the decision to continue on with our palliative care plan. We have been persistent with our team that if G’s condition is fatal, or attempts to “save” him will be futile and associated with severe, long term medical and cognitive consequences where he will not have a quality of life, we do not feel that surgical palliation is the right thing to do. We have not received any judgement from any professional we’ve encountered and have felt 100% supported this entire time.
Last week, I had my last echocardiogram scheduled and though I considered canceling it, I decided to go ahead and go to the appointment. I wanted to make sure nothing had changed, and also wanted the chance to speak with our cardiologist about what to expect for end of life with this condition, and what sorts of things we could consider in making G’s transition peaceful and painless. I was speaking with the sonographer during the exam (she’s scanned me a bunch so we are on friendly terms) about what had happened since we last were in. She was familiar with the reports from St. Joe’s, but felt strongly that she was able to see flow across the atrial septum, which was not the case three weeks prior at St. Joe’s. This was a huge deal because that finding was the major reason we decided not to move forward with gathering more information about surgical options. She asked if we had sought an opinion from another cardiologist in the group who was slightly more experienced with G’s type of heart anomaly. At that point, I decided I would reach out to him to see if he’d be willing to review our images and give us an honest opinion about what he felt was going on, and what, if any, options we had moving forward. Our cardiologist who we had been seeing since the beginning remained guarded in her prognosis, which is a fancy medical way of saying she persisted that baby G had no chance of survival.
Two days later, we were in to see another cardiologist who happens to be the director of perinatal cardiology at the hospital we’ve been receiving care. He gave us his interpretation of the echo that he performed himself that day. He gave us options- a lot of them. I think we probably talked to him for over an hour and his patience and compassion toward us was unlike anything I’ve experienced before- both as an RN and as someone in the receiving end of medical care. We were completely taken aback that we suddenly had options, but with them have come some seriously big and seriously, well, serious, decisions. The kind of decisions where one wrong one literally can be the difference between life and death.
Long story short, he told us that no one, not one person, can tell us right now how our baby will do at birth. For anyone to have given us numbers and statistics at this point is not only unfair, but more than likely untrue. He agrees with our (now) former cardiologist- this is a difficult case. This is not a “textbook” hypoplastic left heart syndrome (HLHS). This is high risk. He has more going on in his heart than a small left ventricle. There is a good chance that when baby is born, he will be unstable. He will present with respiratory difficulties, he will be hemodynamically unstable (heart rate and blood pressure difficulties), and it may be apparent that he is too sick to undergo intervention. An echocardiogram at birth, which can adequately assess far more than a fetal echo can now, could show compromised heart function, and a heart much too sick to be considered for surgery. But, he told us, he could also be born stable. He could be pink, cry, maintain his oxygen saturation and heart rate and blood pressure. He could come out fighting. We had never planned for this scenario because it had never been presented to us as an option. We have been making all of our decisions based on what we’ve been told, and likewise our medical care has been tailored to that as well. We almost chose to be induced two weeks ago, because of some medical conditions I have been dealing with (due to the pregnancy). We ultimately backed out at the last minute because we felt guilty about hastening his entry into and subsequent exit from the world unless I was truly in danger. And here we were, not even two weeks later, talking about the option for our baby to have lifesaving surgery. Talking about the what ifs, what if he is born stable? What if his echo is reassuring and his lungs are not at all compromised? What if he is deemed a good candidate for surgery that could enable him to have a happy and fulfilling life. WHAT IF.
So that day, he asked if we could relocate to Gainseville. They have the best pediatric cardiac surgery program in the state. And they happen to be 146 miles from here and an almost 3 hour drive. Our local facility does not currently have the capability to do cardiac caths or cardiac surgery at the moment, and his recommendation was to be closest to the best facility in the state that does. We told him that it didn’t seem feasible to do that right now, we have no family in the state and two children who need us close by. We said we’d talk about it, though. We decided during that meeting that we would continue with our plan to deliver in St. Pete as close to 38 weeks as possible, assess his stability at birth with a very specific birth plan laid out (we do not want CPR, ECMO, etc. if attempts seem futile and he is really unstable). He told us that they have the ability to transfer him and me to a facility to perform the procedures if we felt it was a good compromise to stay close to home. We left that night feeling comfortable with our “wait and see” plan, knowing now that we’d have the option to transfer him to a tertiary care facility should he be born in a better circumstance than we’ve been planning for all this time.
This past Tuesday, we had a big interdisciplinary meeting in St Pete with cardiologists, CVICU docs, perinatology, palliative care and neonatology. We spoke about the baby, the plan and were able to discuss among all the professionals involved in our case the details as we are getting really close to his birth. They were all on board with our plan, of course, but one doctor did want to reiterate that they do not have the capabilities to provide any of the surgical interventions our baby would need, and while transferring is always possible, it’s still time that he could have been having intervention already at another, better equipped facility. And is it possible that his restricted atrial septum, while not intact, could contribute to him being unstable as the blood not flowing across that atria could cause dangerously low oxygen saturation. I also inquired with our cardiologist about a fetal MRI to assess for something called pulmonary lymphangiectasia which is associated with 100% mortality according to some retrospective studies. So, this morning I had a fetal MRI to assess baby G for that and we will be tasked with making some decisions depending on those results. If he has evidence of it, we may stick with our plan to deliver locally with the expectation that he may not have a good outcome. But, if he doesn’t have evidence of it, Barry and I may be calling in a lot of favors as we explore the option to relocate and deliver at a facility equipped to handle baby G’s needs if he’s born and he beats all the odds that have been stacked against him.
Please send us all your love, thoughts, prayers, positive vibes as we attempt to make the best decisions we can. This has been the biggest whirlwind, emotionally exhausting and taxing situation we’ve ever dealt with. <3