Paxton Finally Gets a Big Boy Heart

Susanne and Scott could not believe that the BIG day was finally here. It had taken the team of doctors so long to even decide what surgery Paxton would be having done. Now there was no turning back. The surgeon had an idea of what he wanted to do, but it would all depend on what Paxton's heart actually looked like once he got in there. Paxton had the most severe form of Ebstein's Anomaly. It was still unknown if he would even be a good candidate for the specific heart surgery known as the cone procedure. If he did end up qualifying for it, Paxton would only be the second child EVER with his type of Ebstein's to get it done. Dr. Del Nido was definitely the man of the hour. It was his time to shine. His masterful hands would hopefully change Paxton's life forever.

Susanne and Scott actually made it to the hospital on time today. They made sure to set three alarms to make sure that what happened yesterday did not happen again this morning. They had to be at the hospital by 7:00am to check in. Moppy and Chief came along for that part of the process. It actually worked in their favor because they were able to spend some extra time with Paxton before he had to head up to the surgery ward. The tentative plan was for the surgery to start somewhere around 8:30am. They knew this would be a long day.

After they checked in, everyone was relocated to the surgery department. Gigi and Poppy met up with them there once Paxton was assigned a bed space. The anesthesiologists stopped by to go over the plan with Scott and Susanne one more time and make sure they did not have any questions. After everyone got to hold Paxton and give him a few words of encouragement, a sedative was given through his G-tube. This made him really sleepy within about ten minutes. Shortly thereafter, Paxton was taken away. Everyone said their goodbyes and off he went. Scott and Susanne had a hard time letting go. They both made sure to hold him extra tight and made sure he knew he was loved. Even though it was painful to hand him over, they knew this was the best decision for Paxton and the appropriate next step in his journey. Nevertheless, they were still scared to death of just the thought of the complexity of the surgery he was about to undergo and the possibility of losing him. All they could do was pray and be hopeful.

After Paxton was out of their sight, everyone was escorted to the family waiting area. They were informed that they would be given updates hourly. This time they did not have a pager with a number to call back to get those reports. Instead, there was a liason between the surgery team and the families who would deliver the updates in person. This sounded better than what they had done in the past for previous surgeries and diagnostic procedures, but it actually made the anxiety level much worse. There were multiple other families waiting in this section of the floor. These families had children that were having surgery as well, but not necessarily heart surgery. Everytime a doctor, nurse, or pretty much anyone that looked official walked by...Scott 's and Susanne 's hearts stopped. They constantly wondered if the person walking by was looking for them to give them news. More times than not, a member from the staff would look in on them and then walk away after realizing they were trying to find someone else. The anticipation and suspense was unbearable.

Susanne had decided to pick a section of the waiting area that had a television because she needed background noise to try to help distract her brain. She remembered all too vividly the first heart surgery when Scott and Susanne were almost driven to the point of insanity with the waiting. Nothing could keep their minds off of Paxton. During the wait, Gigi and Chief took little cat naps, Moppy and Poppy read, but Scott and Susanne were just restless. They couldn't focus on any one thing for too long. The only thing that kept them positive and comforted was the presence of each other.

Time seemed to stand still. It was not going by fast enough. By the time the surgeon made the initial incision, it had been two hours already. What could be taking so long?! Even with Susanne having a deeper understanding of what truly goes on behind the surgery doors, it still felt like it was taking forever. She knew people weren't animals, but she was pretty sure that the set up for surgery had to be comparable. After about another hour and a half later, the surgeon was just getting ready to start the actual open heart surgery. Susanne and Scott appreciated the meticulous nature of the surgeon when it came to gaining access into the chest, but the suspense was unbearable. Right before the surgeon started the procedure, Paxton was placed on a heart and lung bypass machine to externally oxygenate his blood and help keep it flowing through his body while the surgeon worked on his heart. This was the moment Scott and Susanne had to just put their complete trust in God.

After about four hours of actual reconstructive heart surgery, the surgeon was ready to take Paxton off of the bypass machine and see if his heart could function on it's own. This was the moment of truth. Thankfully, all went well. Paxton's new and improved heart was kicking butt so far. Postoperatively, the cardiologist did an ultrasound of Paxton's heart by using a probe that went down his esophagus. This allowed him to get a closer look at how his heart was functioning and to assess how it was handling the changes thus far. The whole cardiology team was more than impressed by what they were able to accomplish. Before surgery, the outlook for what they would be able to do for Paxton was grim. Now, there was more than just a glimmer of hope.

The surgeon ended up doing a multitude of procedures on Paxton's heart.

First, he took down the BT shunt that was placed when Paxton was a month of age, which allowed him to survive up until this point. This procedure was temporarily used to transfer some of the blood going to the body and direct it to the lungs for oxygenation. This was what the cardiology team was waiting for Paxton to outgrow before moving on to the more long term corrective and palliative heart surgery.

Next, they performed the bidirectional Glenn procedure. This was a more permanent type of shunt than what Paxton previously had. In this procedure, the surgeon disconnected the superior vena cava, which was the vessel that carried blood returning from the upper part of the body to the right side of the heart. Then, he redirected its blood flow directly into the pulmonary artery, which takes the blood to the lungs. The inferior vena cava, which was the vessel that carried blood returning from the lower part of the body, was left alone. This portion of blood would still be pushed through the right side of the heart and then out to the lungs through the pulmonary artery to become oxygenated. In essence, this procedure allowed half of the blood to flow through the right side of the heart normally while the other half bypassed the right side of the heart altogether. This was why this procedure was referred to as the one and a half ventricle repair because the left ventricle was functioning normally and doing 100% of its job, but the right ventricle was only doing half of the work of a normal right ventricle.

The third procedure was the most involved. This was called the cone procedure, which had only been performed by three surgeons in the entire world. Dr. Del Nido was one of them. It was the procedure that was specifically designed for people with Ebstein's anomaly. After reviewing Paxton's results from the pre-op tests, the surgeon wasn't sure if he would even be able to perform this surgery on him because his form of Ebstein's was so severe. Basically, the three flaps that were supposed to form Paxton's tricuspid valve never peeled away from the inside lining of the right ventricle during development. As a result, Paxton's right ventircle was small and underdeveloped, whereas the right atrium was enlarged. Due to the lack of a properly functioning valve, Paxton's blood freely flowed back and forth between the two chambers of the right side of the heart. All of these issues combined, prevented any blood from getting to the lungs. This specific surgery was designed to make a functional tricuspid valve using the patient's own tissues. The surgeon's hope was to be able to dissect each individual flap away from the inside wall of the right ventricle. If this was successful, then he would try to suture them together in a way that it would form a cone-like shape. As a result, it would become a functional tricuspid valve. Luckily, his attempts were a success. This was truly amazing given the severity of Paxton's heart condition.

After he tackled the cone procedure, he then decided to remove the portion of the right ventricle that was currently functioning as right atrial tissue. This helped to reduce the heart size greatly.

Lastly, he partially closed the hole that was between the right and left atria, which are the upper chambers of the heart. He left a small portion open to function as a pop off valve to reduce any pressure build up from all of the reconstruction that was done to Paxton's heart.

The end result was miraculous. They were shocked to find that on the postoperative heart ultrasound there was only very mild backflow of the blood through the newly designed tricuspid valve. All in all, Paxton's heart was looking markedly better. Paxton now had a big boy heart.

Later in the evening, everyone was relocated to the waiting room outside of the cardiac intensive care unit. Unfortunately, they had to wait some more before they could see the little guy. The surgeon had talked to them briefly with his assessment. He was beyond pleased with how everything turned out. They were informed that he would be heavily sedated and on a ventilator for the next few days. Trying to wean him off of the ventilator would be the next challenge. All Susanne and Scott cared about at that moment was seeing him. That time finally came and they could finally breathe.