Good evening friends and family. We hope you are in the middle of a fantastic holiday weekend with your families. The last email we wrote, we had told you that Oakes was doing really well and that he had recently been removed from the ventilator. For the following week or so of that email, Oakes continued to exceed everyone's expectations. You can check out the blog, but Becky got some amazing pictures of the Mighty Man. For that week or so, he was smiling, engaging, super alert and very aware of everything going on around him. It was undoubtedly the best condition we had seen him since late May, right before he had the emergency heart surgery on Memorial Day weekend. Becky and I got to hold him numerous times last week and it was totally uplifting to be able to hold him, stare him in the eyes and have him smile back up at us. As abnormal as our lives have been, there was a certain sense of normalcy around last week because he was just doing so well….
But our rollercoaster continued late this past week. Although Oakes was off of the ventilator, he still was receiving a little help from a cannula delivering just a couple liters of oxygen to his lungs. They had weaned him so much and he was tolerating it so well that he was moved to the recovery unit (7 West – literally down the hall from the CICU where he's been since 5/27). But after just barely 36 hours on 7 West, Becky noticed him working a bit harder to breathe. It got worse throughout the day and when the doctors came in, they did not like how much he was "retracting" (the heavy pulling he does under his ribs when he's struggling to breathe). She knew right away, they were going to take him back to the CICU. It was such an emotional letdown for all of us because he was just doing so well we had this false sense of hope that maybe he was going to figure out a way to get home with us….and then completely out of the blue, he turned for the worse. He was struggling enough when they moved him back to the CICU this past Friday, that they decided to intubate him right away. They completed an echocardiogram right away and took some labs. The doctors were concerned enough by the results that they scheduled another cath procedure for first thing Saturday morning (yesterday).
If you recall, the last cath procedure he had did not go well at all. As the doctors were prepping me and Becky for the cath, it was without a doubt, the most direct and the most ominous language they've used with us yet. Dr. Balzer, the head of the cath lab and the doc who performs all of the major cath procedures at St. Louis Children's Hospital (SLCH), came to talk to us before they took Oakes to the cath lab. He said that during the last cath, Oakes' pulmonary veins "spasmed" in a way that he had never seen. He told us that because of that, this procedure was anything but a sure thing. Their intent was to go in, review the blood flow in the heart and lungs and probably balloon all four pulmonary veins. Dr. Balzer also mentioned the possibility of the insertion of stents depending on what they found.
What has been causing the breathing issues all along has been a disease called pulmonary venous stenosis, which is the narrowing of the pulmonary veins (four smaller veins that take blood from lungs back to the heart). It's a brutal disease that gets progressively worse over time and because of the fact that most of these veins exist deep within the tissue of the lungs, there is no operation that exists which can be a quick fix like the operation that Oakes had on 5/28 when they were able to increase the size of his pulmonary arteries (two larger arteries which take blood from the heart out to the lungs). The cath they did on 6/9, scary as it was, actually succeeded in temporarily relieving the narrowing that had occurred in the pulmonary veins, but all of the doctors were very clear that the stenosis would return and continue to worsen. Sure enough, that's what was happening to poor Oakesie the last week – the veins were just getting more and more narrow and his heart was working harder and harder to get blood through it.
As the doctors were assessing Oakes' condition Friday into the afternoon and evening, they began to relay to us that the cath was needed to buy Oakes more time until they can find a proper donor set of lungs. The slight hope that we had held on to around the small percentage chance that there could be a surgical fix before resorting to a transplant was gone – a lung transplant is definitely on the horizon for Mighty Oakes. Although the doctors were clear this was a long shot, it was still a crushing dose of reality for us both.
As Dr. Balzer was going through his final words of wisdom prior to taking Oakes to the cath lab, Becky and I were half listening to him and half staring at Oakes who had been sedated and sleeping since we had arrived at 9am. All of a sudden, with Oakes' head facing both of us, he widely opened his eyes and looked right at us! We immediately thrust our heads down toward him to talk to him. I told him his papa was there and that we loved him. He looked right at me and gave me a big ol' smile! He then looked at Becky and did the same thing. We, of course, started crying. It was like Oakes, the one who should be scared, was the one smiling and calming his mama and papa. It was something we'll never forget. Of course, the doctors didn't like how awake he was, so they gave him a triple shot of sedation medication and within a couple minutes he was back asleep, but what a moment!! It was crazy because in the past when he wakes up, he struggles to open his eyes and kind of opens one before the other, but here, he just opened them both right at the same time.
So the cath went surprisingly quick, maybe three hours all told. Dr. Balzer did indeed insert three stents – one to his lower right pulmonary vein (PV), one to his upper left PV and one to his lower left PV. The venous tissue will ultimately grow in and through these stents which, again, means that the next step has to be a lung transplant. These stents cannot be removed without significant complications. What the stents will do is provide more longer term support, hopefully, for Oakes. They're more permanent and will keep the stenosis at bay, but only where they were inserted; the stenosis will continue elsewhere along his pulmonary veins.
So what happens if we haven't found suitable lungs and the stenosis further down his PVs are causing breathing issues? Then we'd be introduced to the Quadrox machine, the pediatric version. The Quadrox machine is essentially artificial lung support. You will find this hard to believe, but the Quadrox is a small "box" that sits at the foot of the patient's bed and oxygenates the patient's blood. It literally has tubes that run from the heart to the machine and back to the heart, so that the machine is essentially acting as the body's lungs. Is that insane?!??! The pediatric version, we believe, was just publicly released in late April of this year. There have been, we think, three children here at SLCH who have been put on it, including our friends daughter, Elizabeth, just a couple rooms away. The doctors have told us Oakes would be the ideal type of patient for Quadrox, if he, in fact, does need it. This is a bit too intense for me to process just yet, but at least I've introduced it in case it becomes reality. If you're so inclined, here's a link to a picture of the Quadrox - http://www.maquet.com/productPage.aspx?m1=112599774495&languageID=1&titleCountryID=103&productConfigID=126054034616&productGroupID=121118652068 .
Thus far, Oakes has done well since yesterday's cath – he's not retracting, he's responding to his medications and he's been calming down very nicely when he gets agitated. They are very aggressively weaning him off of the ventilator since they believe the stents will better support him this time around. He's still fairly medicated, but is slowly becoming more alert. He's sucking on his ventilator tube as I type. And believe it or not, their goal, as of now, is to extubate him by tomorrow afternoon or evening. They've already reduced his support down to levels that it took more than a week to reduce after the last cath so it appears the stents are doing what they're supposed to do and that Oakes is kicking butt, as usual.
I know I am biased, but I cannot be more proud of my son. He just had his fifth surgical procedure in just over three months and continues to rise to each challenge in front of him. He was not blessed with perfect organs but it's so clear to us that he was blessed with incredible strength and determination and the amazing ability to inspire thousands of people he's never yet met.
Becky's oldest brother, Phillip, got married a week ago Saturday (6/25) in Oklahoma City. Our daughter, Isla, was scheduled to be the flower girl. With everything going on with Oakes, we just didn't know if we could make the wedding or not. Also, I had a big company meeting in Dallas for four days last week. I've not been able to travel much, for obvious reasons, but this was a trip I really had to try to make. So Oakes being Oakes rallies so well the week leading up to the wedding, that Dr. Gazit told Becky he thought she should go to the wedding, so her and Isla flew in to OKC Saturday morning, went to the wedding, had an absolute blast, then flew back Sunday afternoon. Phillip and his new bride, Kat, had a great wedding and the pictures of Isla and her cousins romping around the dance floor were hilarious. Oakes maintained his stability throughout last week allowing me to complete my trip to Dallas. It was literally upon landing that Becky was told he was moving back to the CICU. It was such an awesome gift to his parents – it was as if he was saying "I'll be fine guys, go do your thing." His little body held out just long enough for both of us to be back in town.
Becky's dad, Bill, and stepmom, Jan, helped out this past week (again) while I was out of town and her mom, Connie, and stepdad, Dale, have been here this weekend. Between the four of them, they've completed about 10 home and yard improvement projects around our house that we have had neither the time or the talent (me), or both, to do on our own. People keep dropping off food and groceries and sending cards and prayers and well wishes and emails and texts. You are all too incredible and we never, ever take our friends and family for granted.
In case you do better with pictures vs. my ten thousand words, I've updated the PowerPoint I did a couple emails ago. I also added a great pic of Oakes from earlier last week when Beck gave him a faux hawk; it's priceless.
We will keep you posted, but you may want to subscribe to Becky's blog so that you get updates in between my email novels….Happy 4th everyone!!
Greg, Becky, Isla P. & Mighty Oakes