Four days after the crushing news at the ultrasound, we were at Hollywood Presbyterian Hospital, which functions as a part of USC Medical school. We met with a nurse named Irene who told us that she would be our main contact from here on out. Unlike our imaging specialist doctor, Irene seemed unphased by our tentative diagnosis. NOT unconcerned or unsympathetic- just not unnerved. Irene explained that we would have several different doctors examine Lucas today. We'd even have a specialist who was “blind,” meaning he had zero prior information about Melissa or Lucas, to ensure an objective diagnosis. The first doctor was the “blind” one. His name was Dr. Grubbs, a professor at USC Medical School, and a doctor who specialized in complicated heart defects. Dr. Grubbs was a very tall, heavily built white man, about 40 years old with brown hair and a goatee. He looked more like a guy rent-a-cop than a brilliant doctor, but looks can be deceiving. I'm going to try describe certain people in detail so it's easier for you to get a mental image of them. Otherwise, if you're anything like me, all of these characters will soon begin to blur.
Before Dr. Grubbs started the exam, I could here myself whispering to God. I felt like a scumbag. I felt like one of those people who only calls you when they need something, never just to say hi or to ask how you're doing. The kind of person who calls, and you think “I haven't heard from this guy since he last needed a favor.” Today, I was that guy. I thought about Dr. Nakasha, and the probability of the her having an OFF day and giving an incorrect diagnosis. I thought “while the chances of her being wrong could be 1 in 10,000, it is certainly more likely than Lucas being the 1 in 1,000,000 with this rare defect. So, she must be wrong.” That damn Dr. Nakasha MUST be wrong. When Dr. Grubbs finishes his exam and tells us that Lucas is a healthy baby I'm going to be so upset at Dr. Nakasha for her misdiagnosis. Next, in my silent conversation with God I asked “hey, I've had a good run here. Just give me a couple of months to buy some extra life insurance, let Lucas be 100% healthy like me, then take me instead." I'd be perfectly ok with the idea of God taking me instead of Lucas. It's just not fair to do this to a baby. Such an innocent life. I deserve this way more. All of us do. Like everyone else, I laugh at people when they trip on a crack in the sidewalk and lots of other things like that. Lucas is an innocent soul. He must be spared from paying such a high price.
Dr. Grubbs continued his ultrasound exam of Lucas for about 20 minutes before Dr. Pruetz walked into the exam room and introduced himself. Like Dr. Grubbs, Dr. Pruetz was also about 40 years old. Dr. Pruetz looks more like an accountant than a doctor. White guy, about 40 years old, balding, soft features, short, and well spoken. He had a very kind face, and seemed like the sort of person who you instantly like. Both of the doctors were pointing at blurs on the ultrasound screen. They'd say things to each other like “see the descending arch?” And “let me adjust the contrast mediation to expose the deeper tissue of the aortic valve.” At Washington State University I was a teaching assistant for human anatomy, and I did some post doctoral research on cardiology. I'm very familiar with cardiovascular anatomy. But, I'd always learned about the 99.999% of people who has the same cardiovascular anatomy. If Dr. Nakasha was right about Lucas, his anatomy was all jumbled up and far from normal.
After about 45 minutes of ultrasound examination, Dr. Pruetz excused Dr. Grubbs and continued the exam on his own for another 5 or 10 minutes. Once finished, he invited us to a conference room. Dr. Pruetz gave us a more detailed introduction of himself. As it turns out, he is the director of the fetal cardiology program at Los Angeles Children's Hospital and an assistant professor at USC Medical School. Along with Dr. Pruetz, another doctor joined us in the conference room along with several nurses and our case manager, Irene. Feeling optimistic, I was eagerly anticipating that they would say everything was fine. But, that's not what Dr. Pruetz said. He basically said that Dr. Nakasha was exactly right about everything. That Luke has at least 7 major heart defects, his liver and stomach are in the wrong places, and that his spleen is missing. He went on to say that there are several paths we can choose, and that we don't have all of the information yet. He said the Lucas has “heterotaxy” and that babies with heterotaxy have a much higher than normal rate of other genetic defects such as Down's Syndrome, Edwards Syndrome, Patau Syndrome, Williams Syndrome, Jacobsen Syndrome, Klinefelter Syndrome, velocardiofacial syndrome, and many other syndromes. So, he explained that he doesn't have all of the answers about Lucas YET. I kind of felt like someone kicked me in the stomach. For the first few minutes, I wasn't sure if I was going to vomit. Then I looked at Melissa, and thought about how well we work together and how strong we are as a couple. I thought, “we can do this. It's going to be a battle, but we can do this.”
Dr. Pruetz said “the main concern is Lucas's heart. He has major heart defects that will immediately threaten his survival once he is born. If you decide to terminate the pregnancy, we can help you with that route.” I said “if he can live, then aborting him is not something we are going to do.” Dr. Pruetz replied “well, I can only tell you about the things that I saw in the ultrasound. There could be other things that I didn't see, such as his brain anatomy. An MRI will need to be done to see if his brain is there.” No brain? What the hell? Dr. Pruetz continued “And there is a much higher than normal chance of a chromosomal addition or deletion syndrome, such as the ones that I mentioned earlier. An amniocentesis would have to be performed to determine if that is present. “Oh great” I silently thought. Just when I worked up enough nerve to accept that fact that my son is missing a major organ, his guts are jumbled, and his heart has 7 defects- you add on the chance that his brain may be missing and he may have other unknown problems. Shit shit shit. What else can be thrown upon my son? What kind of a fucked up universe is this? Ted Bundy, a man who was the devil in human form, was blessed with a genius level IQ, good looks, and a perfectly functioning heart- while my son who is the most innocent soul, is burdened with so many major defects.
I asked Dr. Pruetz and everyone else in the room “Am I correct when I say that babies like Lucas are around one in a million.” Dr. Pruetz replied “more like 4 out of a million, but yes you are basically correct.” I said “ok, so you don't really have much experience dealing with kids like Lucas right?” All of the nurses and doctors at once said “oh, no no no- we deal with this all the time.” Puzzled, I said “ok, explain that to me because I'm either not very good at math or I'm not understanding something here.” Irene, our case manager replied “we basically get all of the heterotaxy babies from the west coast and most of the heterotaxy babies west of the rockies. LA Children's Hospital is perfectly equipped to take care of Lucas.” For the first time in a week, I felt at ease. I felt like someone knew what the hell was going on, and I felt like I was in a room full of people who could help us.
Dr. Pruetz said “The most pressing issue is Lucas's heart. When he is born he will basically have no blood flow to his lungs, meaning he'd die soon after he was born if we don't do something. In every developing fetus there is a vessel called the ductus arteriosis that connects the aorta to the pulmonary artery. This vessel normally closes very quickly after a baby is born, resulting in normal anatomy. With Lucas, that vessel MUST stay open, or he'll die. There is no other way for blood to get to his lungs. So, we have to put him on an IV drip of prostaglandins for a few days to keep the ductus arteriosis open” I replied “ok, sounds great. What happens from there?” Dr. Pruetz continued “we'll have to take Lucas into open heart surgery a couple of days after he is born to place a permanent shunt between his aorta and his pulmonary artery so his lungs can have some blood circulation. It's called a Norwood proceedure. Normally, Unoxygenated blood goes from the heart to the lungs to become oxygenated. But, in your son, the vessel that takes unoxygenated blood to the lungs (pulmonary artery) did not form. So, the best we can do is to feed MIXED oxygenated and unoxygenated blood to his lungs. This will result in him looking a bit blue. There really is no other option for this first surgery. Lucas will be blueish purple for the first 6 months of his life because he won't get enough oxygen to his blood. He will get tired VERY easily. For example, feeding will exhaust him.
I asked “If his heart is so messed up, can't we get on the list for a transplant?” Dr. Pruetz replied “well, there are a few reasons why a transplant is not a good choice. The first is that the cavity in Lucas's chest where his heart sits is arranged for his heart, that is flipped 180 degrees and tilted the wrong way. It won't really fit the heart from another person. The second reason is that there aren't a whole lot of babies with healthy hearts ready to donate their hearts. It's just not that common for a baby to die with a healthy heart and be ready to donate. The third reason is that the anit-rejection medications are very toxic and cause many of their own problems. And, the fourth reason is that a transplanted heart lasts about 10 years before it fails. If we do things right with Lucas, and there are no other problems, and we have luck on our side he can live to be 30 or 40 years old with his own heart.” AWESOME! That was the best news I had heard in a long time. Melissa asked “ok, so what happens after 6 months?” Dr. Pruetz said “well, here is where you and Tony have some decisions to make. If we stop with the shunt surgery, and do no more surgeries he will likely live to be about 8 or 9 years old then die of heart failure. If we go on with surgeries, we can do a Glenn procedure when Lucas is about 6 months old. It will take the venous (unoxygenated blood) from the top half of his body and disconnect it from the heart, then reconnect it directly to the lungs, essentially bypassing the heart. This will take a big load off of the heart. Then, when Lucas is about 3 years old we can do a Fontan procedure. That's a procedure where we disconnect the venous (unoxygenated) blood that is returning from the lower half of his body and disconnect it from the heart and connect it directly to the lungs through a Gortex tube. With those three open heart operations, it will basically mean half of his heart is shut down. He will be functioning with only the anatomical left atria and anatomical left ventricle. Although, since Lucas's heart is flipped 180 degrees, it's technically his right atria and right ventricle.” I said “ok, and it sounds like there is another option too.” Dr. Pruetz replied “yes, there is a third option. Basically we can do the Norwood procedure, then create a plan to put some baffles in your son's heart to separate the right and left ventricles, and use some artificial vessels to make the plumbing in Lucas's heart 100% anatomically correct.” I said, “great, let's do that!” Dr. Pruetz looked at me like he wasn't done talking. He replied “we can definitely do that, but it's not without risk. To completely fix his heart it will take at least 5 maybe 6 major open heart surgeries. Each surgery carries some degree of risk for a bleed out, a stroke, a clot, or another major surgical complication. A babies heart is the size of a walnut, so these are very technical surgeries.” I asked him what Dr. Pruetz what he would do if it were his son and he tried to be very diplomatic about it by giving me a soft answer “well, if you are concerned about quality of life, Lucas should have a pretty normal life if we do the Norwood, Glenn, and Fontan proceedures. We've done those procedures many times and they have been done for many years in many countries around the world. If we do the surgeries to completely fix his heart, we'd be stepping into somewhat new territory, even with the best heart surgeons in the world. But, don't become too preoccupied with those decisions. They won't have to be made for almost a year.” I asked him if there are any adults around that I can talk to who were born like Lucas. Could they put me in touch with them? Could I ask them what life has been like?
As it turns out, if Lucas were born in the 1980s or even 1990s, he'd almost certainly die before high school. Today, there is at least a chance that he will live to be 40 years old! That's how much we have advanced in cardiology and cardiovascular surgery since then. I often think about life today in the 2012-2013 era. I think “wow, the 1980s were so much better. No cell phones, less traffic, better music, no school shootings, and we still had Hostess Twinkies. But, Lucas wouldn't have lived if he was a child of the 1980s. That fact changes my perspective.
After our conference with Dr. Pruetz, he shook my hand, looked me in the eye and said “here is my card and my email. Please call or email me if you need anything or have any questions.” I could tell that he meant it. Irene, our case managing nurse, walked with us out of the room. She said “Dr. Miller, your maternal fetal medicine specialist would like to see you now for another ultrasound.” We continued down the hall to the ultrasound exam room, which was a little bit bigger than a standard bedroom (10x10 feet). Dr. Miller started the exam. I sat at Melissa's left side, and Irene stood at Melissa's right side. Dr. Grubbs soon walked in to observe the ultrasound (5 people so far). Evidently, news of a fetal rare cardiac abnormality soon spread to the USC Medical School students because about 5 more people soon joined us. For those of you counting, that makes 10 people in a room with a large examination table, several computer screens and an ultrasound machine the size of a small refrigerator. It was packed. Like the other specialists, Dr. Miller was very nice. He is a white guy and was about 6 feet tall, short blond hair, and had a southern accent. His exam was shorter than the ultrasound exam from the morning. After the exam, he said “I can confirm everything that Dr. Grubbs, Dr. Nakasha, and Dr. Pruetz already said. I know you two have been through a lot this morning and the last several days, and I'm sure you are exhausted. Do you have any questions for me?” I said “yes, I heard that there is a higher than normal chance that Lucas will have some major genetic defects. Do you agree?” Dr. Miller said “yes, that's true. I can tell you that Lucas looks fine so far. There are some things that I would be able to see on the ultrasound which would lead me to suspect some other defects besides heterotaxy and aspleenia, but I don's see those. But, to know for sure we'd have to do an amniocentesis. Do you want to do that?” I looked at Melissa and I could tell that she'd had enough for now. We had been up since 4 am, drove to LA for our appointment at 8am and she'd been poked and prodded for the last few hours. The poor gal needed a break. Besides, I don't think she wanted an audience of ten people when she had a 4 inch needle stuck into her belly to draw out amniotic fluid. So I said “I think we need to take an hour break. We're going to go for a walk, and we'll be back in an hour.”
We walked outside, looked up in the sky and sat on a bench. We didn't really talk. There wasn't much to say. As bad as I felt, I knew she must feel worse. As a woman carrying a baby, you must be so excited about your baby. So excited about what he is going to look like. What will be his favorite color? What will be his favorite toys? When will he learn how to drive? Who will be the first girl he kisses? What kinds of books will he like? All of that kind of got flushed down the toilet when we learned that he might not even survive his first year. As a father, I was crushed too. But, I knew it must be worse for the person carrying the child. I think the first thing I said to her was “want to go get a bottle of water at the 7-11?”
On our way to the 7-11 we talked about our opinions of abortion and agreed that we would only go that route if several independent doctors agreed that Lucas didn't have a brain and that he had zero chance of surviving. We agreed that we would fight for him, we would never give up on any of our children no matter how perfect or imperfect they are, and that we are ready to go “balls out” for him. By the way “balls out” doesn't mean what you think it means. It actually describes a centrifugal governor that controls the speed of an engine by regulating the amount of fuel (or working fluid) admitted, so as to maintain a near constant speed whatever the load or fuel supply conditions. It is most obviously seen on steam engines where it regulates the admission of steam into the cylinder(s).
But, we also agreed that we are done with surprises. We needed as much information as possible about Lucas. If he has Down's Syndrome, that's totally alright. But, we want to know. So, after our 7-11 bottle of water and a lot of talking, returned to see Dr. Miller. Before Dr. Miller drew out the giant needle, I said “I'd prefer it we don't have a small gang of people in here for this procedure. I understand that medical students need to observe to learn, but we have been through a lot and my wife needs some dignity and privacy here.” Dr. Miller understood, and made sure that our request was observed. He told us that there are three types of amniocentesis tests. The FiSH, which yields fast but not complete results, the standard amniocentesis which will show about 95% of possible defects, and the microarray test which takes a long time but will show 99% of possible defects. We told Dr. Miller to do all 3 tests since they can all be done with a single needle puncture and no additional risk to Lucas or Melissa. After the amniocentesis, Dr. Miller advised that we schedule a fetal MRI test in the first week of November 2012 to see if Lucas has normal brain anatomy. We thanked him and left the exam room for a closing conference with our case managing nurse, Irene.
I'm so thankful for Irene. She was so nice to us and answered all of our questions directly to the best of her abilities. Irene told us that Lucas would have to be delivered at Hollywood Presbyterian at around week 38 (near valentine's day). She also scheduled our fetal MRI to determine if there were any brain defects. While we were speaking with Irene, another woman named Karen came into the office to join us. She was very nice and chatty but I couldn't figure out why she was there. After a while I asked her if she was a nurse, and she said “no, I'm a marriage and family therapist. I'm here because you two are dealing with some extremely difficult news and I have been asked to offer my services. If you would like to talk with me, there is no charge to you and it's very normal for couples to have a hard time when they are dealing with something like what you are dealing with. We looked at each other, and I already knew what Melissa was thinking. I said to Karen “I'm so glad that you're here and that you are available. But honestly, having Melissa around is what keeps me sane. She is the source of my strength and I'm just so glad that she is here. She and I get along very well and I know that we can get through this together.” Melissa giggled and added “yes, we are a good team and we are very strong together. We are in this for the long haul and we are going to do everything possible to save Lucas.” As we left the office, Karen gave us her card and added that she is available if we changed our minds.
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