Special Heart: A Journey of Faith, Hope, Courage and Love Read online

  “As I told you yesterday over at Sibley, Paul has some very significant heart issues, so it may take a little while to explain this all to you. So let me start at the beginning,” Dr. Martin said.

  Holding hands, Amy and I looked into each other’s eyes, silently telling one another we loved each other. After a sleepless night, we both did our best to focus on every word Dr. Martin spoke during what surely was going to be the most important meeting of our lives.

  Dr. Martin said that usually when he is called into a situation like he was yesterday at Sibley, there are basically two kinds of congenital heart disease he is looking for: noncritical and critical.

  “With noncritical heart disease, if it is discovered before the baby goes home from the hospital, that’s great. But if not, it’s not going to harm the child. The problem will present itself at some point in time and can be dealt with then: a little hole in the heart or a mild blockage. Whatever it is, it can normally be dealt with down the road,” Dr. Martin said.

  “But when you find critical heart disease, such as the type Paulie has, you must immediately set the wheels in motion,” he said, explaining the activity of transporting Paulie to Children’s the night before. Dr. Martin told us that before he performed an ultrasound of the heart or echocardiogram at Sibley, he had determined a fair amount by simply listening through a stethoscope and physically placing his fingers on Paulie’s chest wall just above his heart—the precordium.

  One of Paulie’s heartbeats, the prominent impulse, was way too strong, Dr. Martin explained. That immediately told him that one of Paulie’s heart chambers was working at too high a pressure and was totally unsustainable. Continuing to listen through the stethoscope, Dr. Martin discovered that along with one chamber working too hard, Paul’s heart sounds were abnormal. He said he could hear that Paulie was missing one of the sounds to his outflow. Dr. Martin drew us a picture to illustrate what he was talking about.

  “In a normal heart that is working the way it’s supposed to, you typically hear a lub-dub sound,” Dr. Martin said. “That second dub sound—if you listen closely enough—is actually two separate sounds,” he explained. “It’s actually a lub-dub-it,” he said.

  Listening to Paulie’s heart, Dr. Martin said he could hear that it was missing the second part of the dub-it. “Typically when that second ‘it’ sound is missing, it means one of three things: the blood vessel is missing altogether, it is blocked, or it’s in the wrong location,” he said. Everything Dr. Martin learned up to that point, he said, was done basically by touching Paulie’s chest with his fingers and listening through the stethoscope.

  Next, Dr. Martin got a more detailed look at Paulie’s heart by performing an imaging test, or echocardiogram. It wasn’t long before he knew exactly what was going on. “I discovered that with Paulie it was a combination of not only that blood vessel being in the wrong location, but also being much smaller than normal,” Dr. Martin said. The technical description of what was going on with Paulie’s heart was “transposition of the great arteries” and something called a “double outlet right ventricle,” Dr. Martin told us. He said the artery that should be going to Paul’s lung was the one that was actually leading to his heart, and the artery going to his heart was the one that should be going to his lung. “Paulie’s blood is flowing entirely in the wrong direction,” Dr. Martin said.

  It was nearly impossible to visualize, let alone comprehend what Dr. Martin was telling us. Although I was scared to death, I was also completely amazed that Paulie’s heart had somehow figured out how to reroute itself for survival. Along with those arteries performing the wrong functions, Dr. Martin said they were also both on the same side of the heart—the right side. Paulie would have to have surgery so they could perform something called an arterial switch to get the arteries back to their proper positions and doing what they were originally intended to do.

  In addition to all that, Dr. Martin explained that Paulie had a few other things going on. “Along with the arteries being in the wrong place, they are also much smaller than they should be,” he said. The technical condition is called pulmonary stenosis. Basically, the artery was pinched to the point where blood was not flowing properly.

  Dr. Martin said Paulie also had something called aortic stenosis, where the aorta is smaller than it should be and makes it very difficult for blood to flow anywhere, let alone to those arteries that were in the wrong place and performing the wrong functions. The blockage in the aorta was also preventing blood from pumping to Paulie’s legs.

  Finally, Dr. Martin said Paulie had two holes in his heart, what he called a VSD and an ASD. He explained that the holes—if that was all that was going on—would not be that big a deal. But in combination with everything else going on in there, they, too, presented a real problem.

  With all those things laid out for us, I was amazed Paulie was actually still here with us. The fact he was alive seemed to indicate, to my untrained eye anyway, that his heart decided to call an audible, improvise, and do whatever it had to do to survive until somebody could come along to fix it.

  Having heard all the complexities going on with Paulie’s heart, I was still dumbfounded that at least some of this wasn’t discovered during any of Amy’s sonograms or checkups, especially since Dr. Martin could basically figure this all out within sixty seconds by using his fingers and a stethoscope. Restraining myself from playing the blame game, all I really wanted to know from Dr. Martin at that point was when could we get Paulie’s heart fixed and who was going to do it.

  Dr. Martin told us Children’s was fortunate to have one of the best surgeons in the world for the type of complicated surgery Paulie would need: Dr. Richard Jonas. He said Dr. Jonas performed more than 350 highly complex surgeries every year, and although Paulie’s combination of issues put him on the extreme high end of the complexity scale, Dr. Martin said he was confident Dr. Jonas could figure out the best way to approach it. I told Dr. Martin we had already done some research on Dr. Jonas, and Amy and I had pretty much agreed he was the guy we wanted working on Paulie’s heart.

  Dr. Martin said his immediate concern with Paulie was something he described as “a ductal-dependant lesion,” meaning Paulie’s life was dependent on the blood vessels being maintained in the open position. If those blood vessels closed up, Paulie could go downhill very quickly, Dr. Martin explained. “The immediate name of the game is to sustain Paulie by keeping the vessels open while we continue running tests to make sure he doesn’t have anything else going on,” he said. Sustaining Paulie and keeping the blood vessels open was primarily the job of the drug PGE1, a prostaglandin that Dr. Martin had put Paulie on over at Sibley.

  PGE1 is in many ways a miracle drug that ensures the blood vessels stay open so as to allow blood to bypass blocked or severely constricted arteries. Dr. Martin explained that when he went through his early medical training, PGE1 didn’t exist. As a result, babies discovered with congenital heart disease in those days had to be operated on almost immediately and always with urgency and drama attached. Now, with PGE1, even though there are always exceptions, it’s very rare that babies need to be operated on in the middle of the night, Dr. Martin said.

  The real miracle of PGE1 is that it gives doctors time to conduct a proper assessment of the baby, looking at the whole child. They are not forced to jump right in and start cutting without having all the information surgeons need or would like to have. “Unfortunately,” Dr. Martin said, “even though PGE1 allows us time to do more sophisticated testing, there are some potential side effects.”

  “What side effects?” I asked.

  “For one, Paulie could stop breathing,” Dr. Martin said.

  Amy and I exchanged quick, worried glances. Dr. Martin explained this was the reason that monitoring Paulie’s breathing, respirations, and blood pressure was so critical right now. Because the PGE1 was doing exactly what it was supposed to be doing, relaxing the blood vessels, Dr. Martin said that if Paulie’s blood pressure dipped below a cer
tain level, there could be a whole other issue of having to give him fluids to bring the pressure back. If the blood pressure dipped too low and stayed there for an extended period of time, it could also cause damage to the kidneys, he explained.

  Dr. Martin said that while everyone, understandably, was focused on Paulie’s heart right now, he didn’t have the luxury of knowing that was the only thing causing him problems. He explained that in cases where babies have heart disease, typically 15 to 20 percent have other organ abnormalities in the brain, the kidney, the spine, or their lungs. “PGE1 allows us to keep Paulie in a safe, stable situation while also giving us time to do a better assessment of what else might be going on with him,” he said. “Even though Paulie has a very, very serious heart condition and definitely needs surgery, he doesn’t need it today or tomorrow,” Dr. Martin said.

  Seeing the dazed looked in our eyes, Dr. Martin seemed to instinctively understand our confusion. “I know this is hitting you hard and without warning, but congenital heart disease is actually the most common birth defect babies have, affecting one out of a hundred live births,” he said. “The truth of the matter is, we have done a very poor job telling the world about heart disease. So most folks, like yourselves, are not prepared for it when they hear it,” Dr. Martin said.

  Truer words have never been spoken.

  Dr. Martin continued to explain that because of the PGE1, whenever Paulie did go into the operating room, he would be completely stabilized and in a much better position to survive the trauma of surgery on his small body. I asked how long, realistically, Paulie could be sustained the way he was on PGE1. Dr. Martin said if he didn’t take a turn for the worse, Paulie could probably be sustained in his current condition for ten days to two weeks.

  “What if Paul does take a bad turn? Will he immediately be taken in for surgery?” I asked.

  The only problem, Dr. Martin said, was that Dr. Jonas happened to be out of the country right now and wouldn’t be back in Washington for another week. I looked over at Amy, and by the look in her eyes I could see she was crushed. After all the stellar information we had received about Dr. Jonas over the past twelve hours, my gut told me Paulie was probably exactly where he needed to be, and Dr. Jonas was the best guy to operate on him. Dr. Martin said Paulie was currently stable, and he thought they could keep him in that condition until Dr. Jonas returned. In the meantime, he said, they would be sharing images of Paulie’s heart with Dr. Jonas while he was out of town so he would be fully briefed upon his return.

  Dr. Martin said that although they pretty much knew everything going on with Paulie’s heart, the only remaining question to be answered was the specific approach Dr. Jonas would take to fix it. He said he really wouldn’t know details on that until Dr. Jonas returned and they could sit down and discuss it. Dr. Martin said one of the things you get with Dr. Jonas is his ability to improvise and be creative. “Once he gets in there, if something pops up that was not altogether clear in the echocardiograms, there’s no one better than Richard Jonas to figure it out,” Dr. Martin said.

  Apart from all the immediate medical questions I was asking, Amy wanted to know about the kind of life Paulie would have after the upcoming surgery. “Will Paulie’s surgery be a one-time fix, or will he need more?” she asked. “Is there a chance he might need a heart transplant down the road? Would Paulie be able to play sports? Will Paulie be able to run around and hang with the other kids in school? Will he be able to play soccer with the other kids?”

  Drs. Martin, Heath, and Stockwell were all very professional and caring as we hit them with our barrage of questions. But after a while it became like some sort of awkward chorus. Every time we asked a variation of our “Will Paulie be able to…” question, what we would hear back was “Don’t know,” “Can’t really say,” “Hard to say,” “No guarantees on that.”

  Amy and I had dozens of more questions about the overall situation and about Paulie’s future, but my overriding specific question had to do with the clock. Would Paulie be able to hang in there long enough for Dr. Jonas to return, or were we taking too big a risk by not sending him somewhere else to have immediate surgery?

  I asked twenty-seven different variations of that one question, but I could not extract a definitive answer. The doctors simply were not in a position, medically or legally, to offer us any guarantees or to tell us what we should do. At that moment, it became clear to me this was going to be our decision—and ours alone.

  I asked if there was anyone else at Children’s who was close to Dr. Jonas’s level of expertise in performing the kind of surgery Paulie needed. Martin said there really wasn’t, but if we didn’t have a comfort level waiting for Dr. Jonas to return, we always had the option of flying Paulie to Boston or Philadelphia.

  “Okay. Let’s say we decide to wait for Dr. Jonas to come back, and then Paulie starts to decline. Will you be able to give us enough of a heads-up so we can get him on a helicopter to Philly or Boston?” I asked. Dr. Martin paused and then answered, “I think we will,” but again, he gave no absolute guarantees.

  As I thought more about the overall situation we found ourselves in, I got even angrier that Paulie’s condition was not discovered during Amy’s pregnancy. If we had been given any kind of advance notice on the baby’s condition, maybe we could have figured out how to keep Dr. Jonas in the country in the first place or possibly had Amy’s delivery date delayed through medication until he got back. A thousand scenarios were racing through my head, including whether Dr. Jonas could get on a plane and come back early from wherever he was.

  I didn’t care what it cost. If we could get Dr. Jonas back earlier, I would happily pay his airfare. Even if we had to send a private plane to pick him up, we would figure that out, too. I would do anything I had to do to keep my son alive. Dr. Martin said he would check to see if Dr. Jonas could come back any sooner.

  By this point, although I was truly appreciative of the doctors’ time and their obvious concern for Paulie, I had grown increasingly frustrated about not getting specific answers to any number of questions we were asking. After several minutes, in a fit of stressed-out, righteous, indignant parental anger and desperation, I kicked into an entirely different nondeferential gear I suspect the doctors didn’t think I had in me.

  “Look, I know this is all legal, and you can’t offer any guarantees. I get that. I am not going to sue anyone. Paulie is our only child. I just need to know one thing. If he were your son, what would you do? Would you send him to Boston or Philadelphia now, or would you wait for Dr. Jonas to get back?”

  It was almost as if I was anchoring a news program with only a few seconds left on the clock and my politician guests had been tiptoeing through the tulips the entire show avoiding giving a direct answer on whether they were going to vote for or against a controversial piece of legislation. To a person, every one of the doctors in this medical lightning round answered, “Wait for Jonas.” “Wait for Jonas.” “Wait for Jonas.”

  Finally! I found the magic key to the mystery lock so I could get the advice we desperately needed. Given the lawyered-up country in which we live, I was thinking this kind of scene probably plays out every day in every hospital in America: doctors knowing what kind of advice they would like to give parents but not feeling free to give it because they put their hospitals and themselves in legal jeopardy if they are too specific and things don’t turn out.

  Amy had gotten very quiet during the later half of the meeting, and I worried that the positive vibe she had from being around Paulie had drained out of her, especially after hearing the scary specifics of what was going on with Paulie’s heart. The fact that we were in the middle of a giant guessing game about Paulie surviving until Dr. Jonas returned didn’t help, either. Amy seemed intensely worried and started to get this vacant look in her eyes.

  Telling the doctors we needed to discuss this by ourselves, Amy and I thanked them for their time and we headed back to Paulie’s bedside. As soon as we arrived in the CICU there
was a commotion going on around Paulie. His A-line had come out again, and the nurses were struggling to get it back in. I had seen the identical thing the night before, but this was Amy’s first time, and she really started to freak out.

  Watching all this unfold, Amy started questioning why it was taking so long to get the line back in and why the nurses were “hurting” Paulie. The nurses, understandably, asked us to temporarily leave Paulie’s bedside so they could fix the line without all the nervousness and second-guessing. So Amy and I left the CICU and went to the waiting room to be with her parents. While there, we filled everyone in on the meeting with the doctors.

  When I got to the part where I explained that Dr. Jonas was overseas, I could see Big Paul was about to blow a gasket. He immediately told me to find out where he could send a private plane so we could get Jonas back. I told him they were already looking into that for us. After a while, I checked back with Dr. Martin and he informed me Dr. Jonas could not come back early because he was giving some very important lectures that couldn’t be canceled, and he was also doing some volunteer work with Project Hope in Asia.

  Amy once told me her father had a favorite expression he often used throughout his career in business: “If you have a problem that can’t be solved with money, then you have a really serious problem,” he would say. Getting Dr. Jonas back to operate on Paulie was apparently not a money issue, so by Big Paul’s calculations this was a really serious problem.

  Growing up, Amy was extremely close to her father. He was always the fixer, the man who could solve any problem that came down the pike. But like the rest of us, Paul Hills couldn’t really do a single thing that was going to change the equation for Paulie. Feeling every bit as frustrated as we were, Big Paul was having a very tough time trying to figure out what he could do to help. After all, his daughter might very well lose her first child. And while Paulie was my first son, he was also Big Paul’s first grandson, not to mention his namesake.