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Authors: DANIEL MUÑOZ

BOOK: Alpha Docs
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As soon as we get an initial bolus (a rapid infusion or surge) of fluid and blood into her, Stella's blood pressure goes up, her heart rate goes down, and her color comes back. The moment we try to lower the rate of the continuous infusion, her vital signs deteriorate. What we're putting in is just barely keeping her even with what's leaking out of her.

Stella is, right now, our sickest patient in the CICU. She has the full attention of the residents, me, the attending, and the nurses, plural. One-to-one nursing isn't enough. At any moment, there are eight to ten things that need to be done. Doctors are issuing orders, asking for readings, directing traffic, with the nurses in a strategic formation, each performing a task.

We are all focused on getting Stella's blood pressure back to normal, and getting it to stay there so that her organs, including her brain, are perfused. The root cause is, for the moment, a secondary concern. We have to stabilize her before we can fix her—resuscitate her first, then figure out how to stop the internal bleeding. And we have to confirm she really is having the kind of bleeding we suspect before we can try to stop it.

We alert the vascular surgery team, because a bleed of this type and severity cannot be dealt with in the cath lab or the CICU, and it cannot wait until tomorrow. When a pipe bursts, you can shut off the water, then open up the ceiling or the floor, and replace the pipe or cap it off. But the human body requires a constant blood flow—which means that a surgeon has to open the body even while the blood is pouring out.

We get ready to roll Stella's bed to the CT scanner in the basement of the hospital to get a noncontrast (no-dye) scan of the abdomen—to determine if there's blood in the retroperitoneum. Given her instability, there's a chance that Stella will “code,” that she'll arrest, and we'll have to do CPR on the spot. We take everything we need with us: the drug box and accoutrements for lifesaving, so I'm ready to run the code, should it occur. Even the ride down is a challenge. We have a woman teetering on the edge of death, rushing to the OR, but to get there, we have to fit all of us and Stella and virtually her whole room—devices, tubes, monitors—into an elevator made for a different era. We roll the bed inside the antiquated box and hit the rear wall, then suck in our guts as the door tries to close but buzzes because the IV pole is sticking out. I tilt it backward and punch the Close button again. The door buzzes a second time, snagging the drawstring of one of the resident's gowns, but finally shuts. Now we just have to hope we don't get stuck between floors. This slapstick routine would be funny if Stella's situation weren't so dire.

Once we get downstairs, the next step is to move Stella onto the CT scan table—while she is still tethered to her devices. We're performing a medical oxymoron, “hurrying carefully”: We're in a rush to get the scan but can't afford collateral damage, a dislodged IV, a line pulled out, or an open port. The transfer, from stretcher to body tray, is like passing a full bowl of boiling soup where the bowl weighs close to three hundred pounds and the soup is life support. Six of us—first-year resident, two nurses, two radiology techs, and me—move Stella, three pushing and three pulling. When we get her halfway onto the table, I see a line get taut, hung up in her gown. I say, “Hold on,” and everybody freezes, her body in midair, while a nurse frees the line. We start moving her again. The whole drill is so counterintuitive: You have to fight the urge to hurry in order to process and check every part, every sign, to make sure nothing goes wrong.

Finally, we get Stella onto the CT scan table and recheck all connections. Every few seconds, I glance at her blood pressure and see that, in spite of the two units of blood, plus medicine to artificially prop up her blood pressure, she's hypotensive. That is, her blood pressure is heading downhill. I tell one of the nurses to get supporting blood pressure meds from the backup bag I prepared in the CICU, just in case. “Just in case” is happening. We plug in the backup bag. The meds are probably the only thing keeping Stella from arresting and dying.

At last, Stella is ready for the scan. We tilt the monitors toward the control rooms behind the glass shield where we watch the results.

Usually, it's challenging for a nonradiologist to follow the CT scan, images flashing on a computer screen in real time as the body passes through the scanner. Unless you do it for a living, spotting an abnormality takes a trained eye. This time it's glaringly obvious. There are massive quantities of blood in Stella's retroperitoneum. She has a volley ball–size mass of blood on her right side. It is, unfortunately, what we suspected.

Next step: Get her out of the CT scanner and back on the bed, which is as potentially disastrous as the first transfer. I phone the CICU, giving my attending the update—massive RP bleed, need the vascular team involved pronto—while I focus through the glass on the team moving her back onto the stretcher. The attending tells me the blood bank called to say they've got two more units of blood, and he and I decide to have them ready to hang once we return to the CICU, about three minutes, elevators willing. We cram in, get the doors closed after the usual buzzing, reach the fifth floor again, resituate Stella in the CICU, and continue to infuse her with fluids to support her blood pressure.

The vascular surgery team shows up just as Stella's son and daughter-in-law arrive. They're visibly distressed at the news of Stella's deterioration. The attending intercepts them for an update while I manage things at the bedside. He explains the situation to the young couple—an unenviable task, given that they thought their mom's condition was serious but not life-threatening. Now she's clinging to life. How did this happen? We can tell the family why the cath made sense and what it showed, but we cannot explain why Stella was one of the unlucky few to experience a catastrophic complication.

Inside the unit, the vascular surgeons look at the CT scan and Stella, and immediately label the case level 1A, the highest rank of emergency surgery. (Level 1 is the highest, but on rare occasions it goes to level 1A.) In terms of the OR, not only does it make the patient next in line, but it renders everyone else—apart from the patients already on the operating table—a secondary concern.

In minutes, Stella is in the OR. The surgeons are not going into the retroperitoneum to evacuate the blood; they're going to the source. They open her leg at the groin level, find the culprit vessel, clip it, stop the bleeding, reattach it, and get out. For somebody this sick, the longer you keep her in the operating room on anesthetics, with an open wound, the worse she's likely to get. The plan is simple: Stop the leak, get out, and hope she's able to tolerate the stress and heal from this life-threatening event.

About an hour after shuttling off to the OR, Stella is back in the cardiac intensive care unit but far from out of the woods. Presumably, the bleeding has stopped, and she's a little more stable. Whether she'll remain that way is the question. Getting a massive inflow of blood and fluids is an unnatural occurrence; when you replace somebody's blood in great volumes, there are a lot of things that need to be replaced along with the blood. We watch and wait and pour electrolytes and clotting factors into Stella as fast as we can.

A side effect of an influx of fluids is enormous swelling throughout the body. This unfortunately causes the pressure in Stella's abdominal compartments to climb to dangerous levels, so high it begins to impede vital blood flow to the tissues in the abdomen. We measure bladder pressure through a catheter, which is a surrogate estimate for intra-abdominal pressure. Normal is fifteen or less. Above twenty-five is abnormal. Hers is over thirty. Those abdominal organs are going to start dying from blood/oxygen deprivation unless we can get the swelling relieved. Unfortunately for Stella, that means back to the OR with the general surgery team.

Thirty-six hours into her nightmare, Stella is on the table, an incision down her abdomen, propped open to provide an outlet for the pressure that has built up. She's now in the SICU, the surgical intensive care unit (one of the few places in the hospital she hasn't been), where the surgeons put on a dressing to cover what would otherwise be her open abdomen. To add to her ignominies, she's intubated, on a ventilator. Twelve hours later—after all kinds of electrolyte abnormalities and acid-based problems—for the first time in forty-eight hours, Stella appears stable. This victory is relative, since it only means that she's not getting worse before our eyes. But there is a glimmer of hope that she might survive.

During the next few days in the surgical ICU, Stella develops what looks to be an acute lung injury from being on the ventilator. The lung problem requires heavier amounts of oxygen through the ventilator, which results in her pulmonary pressure rising to dangerously high levels.

For the moment, Stella is marginally better in terms of her lungs, but her abdomen is still a gaping chasm and the outlook is grim. But each consequent catastrophe has brought her family a step closer to the realities that she, and they, face. In the SICU, the surgical team presents the facts. They're not pessimists, because their nature is to “do,” not watch or wait, but they also deal in reality. This is how it is. This is what can be done. This is how it's likely to turn out. Stella's son says, “She wouldn't want this, hooked up to all kinds of things. I know my mother, and this is not how she'd want to live.” They've had a few days to witness and process Stella's steady decline, and they're suffering with her. Her daughter-in-law says, “Let's just focus on making her comfortable.”

Inevitably, the idea of withdrawing care arises. At first, it's a hypothetical, but gradually it becomes a viable option, then a preferable one. Some people think doctors play God. More often it's the relatives of the patient who make the final call. The ending may be inescapable but the how and when are in the hands of Stella's family. Once they absorb the shock of the impending loss, they have conversations with doctors, with each other, and by phone with relatives who can't be there. In the end, Stella's family decides to withdraw care, opting for “comfort care,” which will hopefully make Stella's passing painless and peaceful. A few hours after their decision, she's gone.

—

Although Stella's family seems to have found peace in their own way, personally I can't shake this case. I've seen death. But I'd never seen a decline this drastic in a patient under my care. A diabetic in the hospital for a routine toe amputation had a precautionary, yet necessary catheterization. Then her blood pressure dropped, and an unexpected internal bleed led to an infusion of fluids, a CT scan, vascular surgery, intubation, and lung complications….The body is not a collection of parts; it's a collaboration that we don't entirely understand. When something major or cataclysmic happens to one part of the body, we can expect other parts to falter—a domino effect.

But that still doesn't fully explain what happened. Or make it okay. Stella's case sticks with me for weeks, a kind of macabre poster child for when everything goes wrong—and quickly. Even in the structured setting of making the right assessments and the right decisions, death is always a possibility. But Stella's case impresses me with the gravity of what would seem to be everyday medical procedures. There is no such thing as a routine test or even a routine decision. Now every time I order any test or procedure for a patient, I take a moment to envision the worst-case scenario. What if this patient has the most serious complications imaginable? Will I be able to look back and say that ordering the test was still the right thing to do? If the answer is no, I have no business ordering the procedure. In Stella's case, the answer was and still is yes. She needed a catheterization, and faced with the same circumstance, we'd send her to cath again. But now, and perhaps forever, whenever I see a patient, I am reminded of the importance, before I order anything, of asking, “What if…?”

12
ROTATION: HEART FAILURE AND HEART TRANSPLANTATION, PART II
As a Sort-of Veteran

From now until early February, I have my second rotation of heart failure, but this time I'm not as awestruck. It's a cold morning in the dead of winter, but somehow the short days and dark skies lend an appropriate gravity to what I'll be a part of over the next two weeks: determining who's eligible for new hearts, trying to delay or even reverse death sentences, ironically by installing organs from the dying to keep the recipients living—trading one life for another.

On my drive to Hopkins downtown, I think back to my first heart failure rotation and my concern about the “grayness” of the ethical dilemmas involved. What keeps us from dwelling on the “should we or shouldn't we” issue is that most of the time, we can't. There are only a few dozen transplants a year at Hopkins, which means that a lot of people on the list will not get a new heart. Most patients will be treated some other way. The unlucky ones simply won't survive.

The mental tug-of-war starts when somebody does get on the list. As Fellows, we are being trained to do what we can to heal the patient. But what does “healing” really mean? Keeping the patient alive? Guaranteeing a certain quality of life? Should we aim for a better quality of life for a short period or accept a lesser quality because it might mean living for longer? There is no single, conclusive metric we can rely on.

By the time I get to the hospital, my mind-set is back in full heart failure mode. Having looked at the previous Fellow's patient list, I'm ready to make rounds, to see the three patient types: the nos (those who don't qualify for the “new heart list”), the yeses (those who are on the list or will soon make it onto the list), and the vets (those who've gotten a heart and are back for a checkup and/or problem).

I confer with the attending, Dr. Dwight. Only in his midforties—young for a senior heart failure attending—Dr. Dwight is soft-spoken, calm, unflappable yet fully engaged. He doesn't have a powerful presence. Instead, he's a wise and gentle teacher. He gives me autonomy, but I can sense he's paying attention to everything that I do.

One of the things I notice as we go through rotations is that Dr. Dwight is never excitable or celebratory about a transplant. He accepts that, even in successful cases, the patient is likely to have problems, that he or she will still struggle through life. He knows that the reality is not as simple as getting a brand-new heart and a brand-new carefree life to match. He's a realist. I see seven or eight patients a day, and Dr. Dwight's style reinforces a steady, been there, done that rhythm to my rounds. His affect seems to constantly convey, “Nothing is happening that in some way I haven't seen before.” Personally, I have not yet attained that perspective.

Medicine often does a good job of natural casting. If there's a time you want a hotshot—all adrenaline and guts—who believes he can do anything, it's when an ambulance delivers an almost-flatline patient to the ER, or when you send a patient to the OR for a lifesaving operation. As a Fellow, it's sort of exhilarating to be around that kind of cowboy. But for the long-term well-being of the patients, once the heroics are over, they don't need high fives; they need wisdom and care. That's Dr. Dwight.

—

Toward the end of my first week, a forty-two-year-old man named Cliff shows up. He's a high school history teacher in southern Delaware who received a new heart three years ago—in other words, a vet. If it weren't for his chart, though, I'd never have guessed he'd had a transplant. He's not only a history teacher but a basketball coach, and he's still fit enough to play one-on-one with his kids. But according to Cliff's charts, when he suddenly began having chest pain on a winter night three years ago, EKG and blood tests revealed that he was in the throes of a heart attack so massive that it would have floored anyone in subpar shape. He was put on a breathing machine and hooked up to every kind of life-support medicine, then shipped to Hopkins. The heart attack was so cataclysmic that Cliff was unable to come off life support, because he had barely enough heart cells to keep him alive. And it all came without warning to a nonsmoker with a good diet, no previous heart attack, no prior evidence of cardiovascular disease (other than family genes—father and uncle had had middle-aged heart attacks). To get him off life support, Cliff had gotten an LVAD, or left ventricular assist device, an implanted pump that bypasses the squeezing chamber of the heart and reinserts into the aorta to provide a temporary engine.

While an LVAD can be put in as a temporary solution for a patient waiting for a transplant, in Cliff's case it was put in as essentially the only solution to save his life in the immediate term. There was no way of telling whether a transplant would be an option. A patient can walk out of the hospital with an LVAD, but it's an imperfect solution since it has a driveline that comes out of the body to connect to an exterior battery pack. After a few months, Cliff's driveline got infected. He came in, was treated with antibiotics, had the pus drained, went home, got infected again, came back, was treated again. Because Cliff's driveline was supplying the electricity needed to run the LVAD and his heart, there was no way to take it out, clean the entry point, even find another entry, and put in a new line. Removing the driveline and LVAD would likely kill him. Meantime, the infection was winning, and if Cliff didn't get a new heart soon, it would eventually kill him. Fortunately, after three months on the list, Cliff received a transplant, and had been doing well…until now.

Like a good coach, Cliff has played by the rules since he got a new heart. He has taken his meds and stuck to his diet. To keep his immune system in check, he has also followed his immunosuppressive regimen, which unfortunately leaves him more susceptible to infection. He came in for his scheduled heart biopsies, routine visits to look for any microscopic evidence of rejection.

Still, a few weeks ago, Cliff was hospitalized in his hometown with diarrhea, nausea, and general gastrointestinal upset. Ordinarily, patients with diarrhea and dehydration would be sent to bed, told to call in sick to work, load up on liquids, and rest until it's over, but the threshold for caution is lower in transplant patients, primarily because they're on an immunosuppressive regimen. As a result, they're vulnerable. They're taught to be proactive and to report their symptoms early. And doctors are inclined to send them to the hospital. Better to bring such patients in and ultimately find nothing than to not bring them in and miss something important.

With only GI symptoms, Cliff would have been on a regular floor—ordinary problems indicate ordinary treatment. In fact, they teach you early in med school: “When you hear hoofbeats, look for horses, not zebras.” Look for the obvious or ordinary and treat it as such. But given Cliff's cardiac history, the situation actually requires a check for zebras, for something out of the ordinary. During my examination, Cliff mentions that his stomach has been bothering him; the diarrhea and a light fever are making him feel “kinda punky.” Cliff says, “Doc, I don't know if I have to be here, but I figured I'd tell you guys about these symptoms.”

Based on his blood tests and abdominal imaging studies, I'm inclined to agree that this is probably just a garden-variety stomach bug. I confer with Dr. Dwight, who agrees with my take and treatment plan. After Cliff has spent a couple of days in the hospital, we've rehydrated him; he's keeping food down and is feeling a little better. We send blood to the lab to check for the presence of certain viruses, ones that most people could carry without even minor symptoms but that, in an immunosuppressed patient, can wreak havoc. The specific virus we're hunting is CMV, or cytomegalovirus, because in the right host, it can cause systemic (generalized) and organ-specific illness—pneumonia, gastritis, colitis. We check for the presence of viral copies (the number or concentration of a particular virus within the blood). We send a sample of blood to the lab, and the technicians try, through DNA replication techniques, to grow CMV. The results come back: Cliff's blood CMV-PCR (polymerase chain reaction) is negative. And, importantly, his symptoms are improving.

Despite our ingrained caution, this case appears to be a plain old stomach bug. After two more days of keeping his meals down, Cliff says, “I want to get out of here. Sorry to have taken your time.” We reassure him that this was the right thing to do. Dr. Dwight and I send him home with a caveat: “If you're not completely better in the next few days, come back.”

—

During the second week of the rotation, Cliff calls to tell us that his symptoms have reappeared. We know how reluctant Cliff is to return to the hospital, so this problem is real. I suggest that it might be time to do a colonoscopy, and Dr. Dwight nods. There are conditions that can hide in the colon—CMV being one—that can infect it without being detectable in the bloodstream. We hadn't kept on pursuing CMV before because Cliff had shown signs of getting better. Now that he's sick again (or still sick), it's time to be sure.

I call the GI team and say, “We've got a transplant patient, and we want a colonoscopy—concern for possible CMV colitis. Can you do him tomorrow?” The GI team agrees. Prior to the procedure, I tell Cliff to hydrate at home. If the endoscopy schedule had been jammed, we'd have kept Cliff home longer.

We have Cliff get prepped for a colonoscopy, which means drinking a gallon of fluid ironically named “GoLYTELY.” The “go” part is accurate; the “lightly” part is advertising. It's supposedly flavored—ginger ale or lime or cherry—but the truth is, it reportedly tastes awful. Crucially, it cleans out the colon so that the GI docs can have a clearer view and take biopsies if necessary in their hunt for CMV.

Cliff's treatment team now consists of me; Dr. Dwight; an attending-level gastroenterologist for the procedure; and because of the nature of CMV colitis, Dr. Williams, an attending who specializes in infectious diseases. Dr. Williams tends to recommend an IV formulation of an antiviral medicine, valganciclovir, which will hypertarget the CMV. But dosing it is tricky—it needs to be tailored precisely to the patient's age, weight, and kidney function.

The GI team does the colonoscopy, and, sure enough, Cliff has erythematous (reddened and irritated) patches on the inner lining of his colon. The GI docs biopsy the areas and have them stained for CMV, among other potential culprits. Results usually take twenty-four hours, but we want to know sooner. I contact a colleague over in the pathology lab, and he confirms the preliminary results that Cliff's biopsies tested positive for CMV. And this time, when we repeat the blood work, the CMV shows up there too.

Although CMV isn't immediately life-threatening, the patient's substantial discomfort means that the sooner we can start the IV treatment, the sooner Cliff can begin to feel better.

On Monday, we put in a PICC line, or peripherally inserted central catheter, which goes in like a regular IV but snakes into a deep vessel, feeding medicine to Cliff's superior vena cava. While a standard hospital IV can fall out and lasts about three days maximum, the PICC line is a longer-term IV that a patient can go home with. It also permits administration of powerful medications into bigger, central blood vessels—medications that can be caustic to smaller, peripheral blood vessels that regular IVs go into.

Before Cliff leaves, a nurse walks him and his wife through the home-care routine for the valganciclovir. He'll need to take it once a day in a half-hour infusion. The other twenty-three and a half hours of the day, he'll be comfortable and at home, where there's less chance of picking up some other infection. And a visiting nurse will come to Cliff's home several times per week to make sure he's doing it right.

In the same way that it's difficult to definitively identify CMV, it can be hard to definitively figure out when it's gone. Cliff's symptoms should get better within six or seven days of starting treatment. To assure that the infection doesn't recur, he will need to come back to the transplant infectious disease clinic and the heart failure clinic over the next few weeks. I keep tabs on him through the electronic medical records, and conversation, and it seems that he's doing better. But Cliff will always be a transplant patient, always vulnerable, with doctors who are appropriately paranoid, looking for the next threat.

Cliff is a perfect case in point that the typical heart transplant patient's life, after the initial miracle, isn't dramatic; it's actually dull and monotonous, meticulously monitoring everything, most of which turns out to be nothing, but which could be something. A transplant isn't a cure; it's a stall, a walk through a minefield, slow and tedious, even boring…unless something blows up. For Cliff, this time it was CMV. Next time it will be something else—a respiratory infection, a cut that doesn't heal, a urinary infection. The rest of his life, which we hope is a long one, will require a proactive, risk-averse attitude on the part of Cliff and his doctors. Not only will we tolerate hypochondria, we will encourage it. Overreacting is good. Boring is good. It means a longer life.

—

That Friday, I leave around 6:00 p.m. For heart failure, it's been an instructive but unremarkable day. Cliff went home. And he's immediately replaced by other Cliffs.

An hour later, I'm having sushi when my beeper goes off. It's a message to call a Mr. Mann about his son Richie. Mr. Mann asks, “Is this the heart failure doctor at Hopkins?” I say it is, and he explains that Richie is an outpatient of Dr. James, an attending on the heart failure service. In a very calm voice, Mr. Mann tells me that Richie's defibrillator has gone off eight times in the last hour, as if he were mentioning they had to reboot their computer. I start scribbling information on my place mat. A very young man with an implanted defibrillator (an internal device that delivers electric shocks to the heart to reestablish normal sinus rhythm) is hardly routine. So when Mr. Mann asks, “Is this something we need to worry about?” I'm already thinking “Worry? How about panic?” but tell him that he should call 911 and/or proceed to the nearest emergency room and that I'll get back to him shortly.

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