Friends and family, good evening. We hope you are all happy and enjoying your families this weekend!
This is far and away the toughest email I’ve written and I’ve been struggling with how to even start it. I guess first things first – Oakes is fine right now and in pretty stable condition. But he is sicker than we or his doctors ever knew and continues to amaze us with his strength.
Let’s start back when Becky wrote the email below, last Wednesday. We were in Oakes’ room at the CICU waiting for this catheter procedure. That procedure didn’t take place Wednesday due to a mix up, but we had a consultation with Oakes’ cardiologist, Dr. Caroline Lee. She wanted to let us know that on the echocardiogram that they had performed on Oakes the previous day, they saw a couple of things that led the staff to believe that there may be some pulmonary stenosis (for us laymen, stenosis refers to the “narrowing” of the veins) present in Oakes’ pulmonary veins that bring oxygenated blood from each lung back to his heart. So the echo on Thursday would determine if that was indeed the case and, if so, how pronounced it was and they may intervene. If they were to intervene, that would likely include ballooning these pulmonary veins (similar to what they had done during his May 4th to his pulmonary “branch” arteries which deliver blood from the heart to the lungs). The problem is, these pulmonary veins are much smaller than the “branch” arteries which makes it more difficult to balloon or to insert a stent.
Thursday, we arrived around 9am with Oakes’ cath surgery scheduled for around 1pm. This was not foreign to us, since we had arrived to St. Louis Children’s Hospital several other mornings expecting a surgery that day. Oakes had several doctors and nurses come through that morning, which was nothing unusual for a surgery day. Dr. Murphy, who performed the 5/4 cath and who was about to perform the this cath, stopped by to say hello as well. Oakes finally got carted out of the CICU around 2pm. We were told it’d be a 3-4 hour procedure, so we grabbed a late lunch and just hung out in Oakes’ CICU room.
Around 4:30pm, Dr. Lee came in to the room to give us an update. Unlike every other time we’ve talked to her, she suggested that we go to the consultation room of the CICU. I didn’t really think anything of it at the time. There was another family in the consultation room, so we sat down in a large conference room behind the main nurse station that the nurses sometimes use as a lunch room. Dr. Lee has been Oakes’ cardiologist since October when the Truncus Arteriosus was first diagnosed. We’ve developed a great rapport with her and we can tell she really loves the little Oakes man (she also loves my wife, but who doesn’t?). Dr. Lee sits us down at the far end of a 20-person conference table. She has piece of paper with a diagram of a heart on it and a pen in her hand. When I made eye contact with her, I could tell she was uncomfortable. “It’s not good,” she says after a deep exhale. Becky, already knowing the gravity of the situation, asked if Oakes was still alive. “He is, but it’s really not good,” Dr. Lee responded. My heart sank like I’d just been punched in the throat and Becky started to cry. This was not news we were ever, ever expecting to hear. It just wasn’t. Dr. Lee told us that his veins were so small, there was no hope in ballooning them or stenting them for a long term fix and that he was not reacting well to the cath. She said she’d be back with an update. Beck and I walked like zombies in disbelief to Oakes’ empty CICU room and just bawled uncontrollably.
They had taken the catheter through an artery in his groin up to his pulmonary veins. What they realized was that only one of the four pulmonary veins (two on the left and two on the right) were letting ANY blood back to the heart. This was shocking to all of the doctors – they had NEVER ONCE seen pulmonary vein stenosis (again, narrowing) with Truncus Arteriosus and had not seen anything on any previous echocardiogram or cath or x-ray or blood test to tell them this was occurring. On the echocardiogram they did on Tuesday, they had seen what they thought was some congestion on one of the left pulmonary veins. So they took the cath directly to that vein in hopes of relieving the congestion. When they went to balloon that vein, Oakes went into cardiac arrest. This immediately signaled to the doctors that what they saw on the echocardiogram was not congestion, but rather was relatively normal blood flow – unfortunately, it was the ONLY blood flow coming from the lungs to the heart. So when they essentially “blocked” that vein with the balloon, Oakes had no blood flowing to his heart. Dr. Murphy, of course, immediately pulled the balloon out, but Oakes was reeling…..this was about the time Dr. Lee gave us the first update.
About 30 minutes later, Dr. Lee came back for another update. Somehow, things had gotten worse and Oakes was in really bad shape. He needed to be put on an ECMO machine (extracorporeal membrane oxygenation) which is essentially a life support machine. Dr. Lee said this would buy us some time to call family to say goodbye to Oakes because things were that bad and he probably wouldn’t come off the ECMO machine. We were just stunned and totally crushed. Somehow Becky got up the energy to call her family and tell them to get to St. Louis as quick as they could. I called my parents who were visiting my sister in Des Moines to tell them the news. Within an hour, we had almost all of our parents and siblings driving in from Oklahoma City, Tulsa, Des Moines and Wilmington, NC (Becky’s sister, Christina was already here from DC). Our families were, of course, shocked by this news since Oakes had been so stable, relatively speaking, since his emergency heart surgery on 5/28.
I can’t really describe exactly how we were feeling and I hope none of you ever have to experience anything remotely close to it, but to put it simply, we were just a mess.
Dr. Murphy, the surgeon who performed the cath, then came by and took us into the same large conference room that Dr. Lee had us in earlier. He explained some of the more technical details of what they found and was very empathetic. We actually got a bit of good news from Dr. Murphy – Oakes had stabilized on his own and was not put on the ECMO machine. This was, again, a stunning development since we had heard from Dr. Lee just 45 minutes or so earlier that he may not make it and that ECMO was the last resort to save him. The rollercoaster continued…
Shortly after talking to Dr. Murphy, still spontaneously breaking down whenever we would try to think about what had been communicated to us, a team of doctors and nurses wheeled Oakes into his CICU room. He was indeed not on the ECMO machine and it just broke our hearts to see the little man, knowing what he had just been through. But the important part was that he was stable, but we didn’t know how long that would last.
Over the next few hours we talked again to Dr. Lee and to Dr. Gazit, the Director of the CICU. The story that was forming was that the narrowing of the veins were so bad and went so deep into the lungs (from the heart) that it was irreparable. Leave it to Oakes – not just the truncus, but also narrowing of the branch arteries; not just tracheal rings, but complete tracheal rings that went the length of his trachea; not just narrowing of the pulmonary veins but intense narrowing the entire length of the veins. They don’t when this narrowing occurred – if he was born with it or if it developed from some infection along the way – and explained that they didn’t diagnose it until now because it was so rare and had never been seen in conjunction with truncus arteriosus. Any issues Oakes was having were assumed to be related to the significant heart issues he had, so previous echocardiograms and other tests weren’t really focused on the pulmonary veins.
The other thing that was clear was the only chance Oakes had at survival was a transplant. Not a heart transplant, but a double-lung transplant. The heart is functioning as it should, but the severe stenosis in the pulmonary veins has put the lungs in bad shape. The lung transplant would include a new set of pulmonary veins. Nerd that I am, I have attached a PowerPoint with pictures to (hopefully) make things easier to understand
So now what?
Now we study up on lung transplants and hope Oakes stays stable as can be. We’ve been told he should qualify for a transplant in spite of his heart issues – since they’ve all been repaired and it is functioning well, it’s not held against him. We were given a 67-page packet Saturday that tells us everything we’d ever want to know about pediatric lung transplants. Once we review that, we’ll get a chance to sit down with the transplant nurse so she can answer questions and ask us some herself. Additionally, they’ll be running some blood tests on Oakes to ensure that all of his other organs are properly functioning and that there is nothing which would disqualify him from a transplant.
Here is the reality of what we’re trying to absorb – around 50% of pediatric lung transplant recipients do not make it to five years past surgery. That number dips to around 30% ten years out. And the staff has been really, really clear with us – the transplant is NOT a cure, it is a treatment. A lung transplant and the complications afterward are much more difficult than even a heart transplant, which boggles our minds.
Miraculously, we live in St. Louis and Oakes is in St. Louis Children’s Hospital. Would you believe that St. Louis Children’s Hospital has performed more than 350 lung and heart/lung transplants since 1990 making it the most active pediatric lung transplant program in the world? And would you believe that Dr. Huddleston, Oakes’ cardiothoracic surgeon, has probably performed more pediatric lung transplants than anyone in the world? I figured you’d believe that, but it’s still miraculous to us.
If all goes well, we can have Oakes on the transplant list by Friday. The average wait to find donor lungs is about 2-3 months for a baby Oakes’ age (they’re much more in supply than for a teenager with cystic fibrosis, for instance). It’s critical that Oakes stays stable throughout this entire process. If he regresses significantly and needs to go on ECMO, he will be disqualified for a transplant.
As you can imagine, our brains are not absorbing this painful information completely yet. We’re doing our best to stay strong for little Isla P., but I’d be lying to you if I said it was easy. The rush of emotions that come over me and Becky when we’re snuggling with Isla or reading her books at night are too intense to describe considering what is going on with Oakes and what may or may not happen with him. We’re just trying to take it one day at a time, as hard as that is right now.
Wondering why I am sending this out at 5:30am Sunday morning? As “unluck” would have it, Becky experienced some intense pain this evening after dinner and I won’t bore you with the infamous “details” here, but we’re sitting in Missouri Baptist Hospital and she’s about to have her appendix removed. No, I’m not kidding. She will be here until Monday morning some time. We’re waiting for Ashton Kutcher to jump out from behind a curtain or something. I’ll try to keep you posted on her surgery too (no news on that should be considered good news).
I know we are in your thoughts and prayers, so please keep us there for a long time. If you’re inclined to be an organ donor, go for it..…little Oakes wouldn’t have any shot at all without it. I’m the first one who needs to change the checked box on my driver’s license. I know we’ve heard it all before, but it’s put into unique perspective at times like these – just appreciate every single day with your family.
We love you all.
Greg, Becky, Isla P. and Mighty Oakes
www.babyortyl.blogspot.com
Hanging out at the airport with 
Around and around they went!
Isla having a hollywood moment!
