The surface of the heart is warm, moist, a little slimy. It brings to mind the sticky mucous covering of a night crawler, like a thin coating of oil. Transformative energy flows from its imperceptible electrical charge, providing a spiritual awakening for the exploring finger. A reflexive jump instigated by touch results in a momentary stillness; then its constant rhythm—“la-lup”—quickly resumes.
Through a stethoscope, the couplet sounds crisp, with snaps as the valves inside open and close in synchrony. The path of electrical energy originates in a small clump of cells coursing through the heart, flowing along cables and morphing into a neon green line across a flat-screen monitor: a straight, horizontal section broken with a small hill, then a series of jagged up and down peaks followed by a larger hill as the electrical charge moves down the heart. After a quick rest, the landscape repeats. This whole complex creates a single beat of the heart.
My identity as a physician took form as I wandered into the field of anesthesiology. But it was not completed until I found my passion in working with children—and the heart. And so I evolved into caring for children with sick or deformed hearts.
JOHN WAS A HIGH SCHOOL sophomore and dreamed of playing basketball. He loved the deep, resonant thud of leather bouncing off wood and echoing through the hall; the screech of athletic shoes on the floor; the sight of the ball sailing through the air, its seams revolving like a world of its own; and the sound it made passing through the rim—nothing but net, that distinct swish—before falling to the floor. He was about to get his shot—again. By his mother’s account, John had been the last player cut from the team his freshman year. Now, a year later, with hard work and determination, he would realize his dream of making the team. After all, Michael Jordan had been cut from his high school team too.
Tryouts turned into a nightmare before warm-ups were completed. John’s body couldn’t deliver on the enthusiasm of his mind. His legs failed to perform to the level of his determination. Running up and down the court, John lagged. Another player dribbled the ball right around him. John’s legs felt leaden. Lacking speed or energy, he was losing hold of his dream, and he knew it. His anxiety grew as, the harder he tried, the more he gasped for air. Something was wrong.
He told his parents about his trouble and soon found himself speaking with his pediatrician, who referred him to a pediatric heart specialist, who referred him for cardiac catheterization to determine the cause of his failing energy. I provided his anesthesia care when he presented for a diagnostic procedure.
I spoke with John and his mother before the procedure, which would require a trip to the cardiac cath lab. After examining him, I sensed that his basketball season was finished. Indeed, my concern was no longer for this season, or the next, but for whether John would ever play basketball again.
He rested comfortably on the cart, and his health history was unremarkable, aside from the fatigue and shortness of breath. On physical exam, John was a little chunkier than I expected for a basketball wannabe. His belly was fuller than anticipated. This wasn’t fat; he was overweight from fluid retention. His heart, unable to keep up with the returning blood, was causing his liver to engorge, pushing his belly out. Excess fluid filled his belly and caused his legs to swell. Not dramatically, but any swelling was problematic. I came to my own diagnosis but didn’t mention it. It was neither definitive nor mine to make, so I limited my discussion to the intended anesthesia care. But it was clear to me that John’s heart was failing and he was in trouble.
John’s proposed trip to the cardiac cath lab was not intended to provide therapy. There were no clogged arteries needing to be widened with a stent, no incompetent or narrowed heart valves needing to be repaired or replaced, no holes between chambers needing to be shut. This procedure was meant to provide nothing beyond a diagnosis, and the cath lab was the diagnostic tool of choice.
The cardiac cath lab is a cold place. It is chaos on a tether. I have entered this room hundreds of times and always find little evidence of cohesion. The patient entrance opens to an expanse of vacant space lacking a discernible purpose. Halfway across the room is a floor-to-ceiling mass of technology that appears unnecessarily crowded. On the inverted maze of runways that constitutes the ceiling, a series of beams, bars, pipes, and cables crisscross above an anchored procedure table. From the beams drop the booms supporting large fluoroscopic—X-ray—tubes that rest to either side and above and below the procedure table. A row of flat-screen monitors, in constant territorial conflict with the fluoro units, hang from the ceiling.
Every piece of equipment fights to be the closest to the procedure table: the anesthesia machine, attached gas lines, monitors with electrical cords, an anesthesia supply cart, a defibrillator cart (to deliver an electric shock to an erratically beating heart), an ultrasound machine that uses sound waves to spy harmlessly inside the body, and a multipurpose cath cart. Around the table are the poles holding IVs and infusion pumps that supply drugs to the patient. The cords and tubes coursing from the procedure table present an unfortunate risk for tripping.
This place is a danger to head and foot. In this room, if I collapsed in my anesthesia nest, there would not be enough space for me to land on the ground.
Above the maze of ceiling structures sit can lights whose rays, in a failed attempt to light the room, are broken by the vast array of suspended equipment and instead cast shadows in all directions. The patient on the table can easily seem lost in this jungle of technology. Murals rest high on the walls near the ceiling, attempting to soften the room with the image of a biplane soaring through white, billowy clouds while a hot-air balloon and a kite float nearby. But the view is obstructed, and the patient would need to strain to see it.
WHEN JOHN’S GURNEY WAS PUSHED to the procedure table, his hand cautiously fingered the edge of the table, and he began to slowly transfer himself. As he moved, he needed to avoid hitting his head on the fluoro unit hovering within striking range above the table. The effort he exerted confirmed my belief that his heart was failing. I noted his energy deficit as he moved. I needed to consider the extent of his heart disease, and whether it was more involved than I believed, to determine whether my plan for anesthesia should be revised, and whether my intended drugs needed to provide more protection for his heart.
A failing heart and the induction of anesthesia are diametrically opposed. Drugs used to induce anesthesia depress the heart and can tip a patient like John over the edge. In an anesthetized state, his heart might not be able to beat strongly enough to provide adequate blood flow or pressure. That is, John could go into shock or suffer cardiac arrest. John presented me with the precarious job of striking the right balance between providing comfort and maintaining sufficient function in an already underperforming heart.
I considered making changes to my plan to prevent drops in blood pressure or heart rate. But for every pro there is a con, and my second-choice drug—the drug I would select if I thought his heart was critically failing—can cause unpleasant hallucinations.
When John centered himself on the procedure table and lay flat, he appeared comfortable. My mind eased because a poorly functioning heart would not allow such a level of comfort. His breathing would be too labored as his liver pushed on his lungs, already congested with excess blood. Rather than being anxious, I was vigilant. (An anxious anesthesiologist is prone to making poor decisions.) With the drugs from my original plan, John safely entered the state of anesthesia.
After the preparations for the procedure were completed, I stepped back from the table to look at John, lost under the blue sterile procedure sheets. A hole in the sheets maybe five inches across exposed his right groin, giving access to his blood vessels. It was the only patch of skin in sight, the only clue that a teenager was actually present. I was connected to John solely by the sounds from the monitor calling out his heart rate and oxygenation, with the lines of his well-being crossing the monitor screens. It was easy to lose sight of the fact that this was more than a sick heart—th
at a whole life lay in the balance.
A biopsy forceps, a wire several feet in length with the tip holding two tiny articulated cups that snap shut and grab a piece of heart not much larger than the head of a pin, entered John’s femoral vein through a needle and catheter placed in his right groin, then snaked up and into his heart. Little chunks of heart muscle were then snipped from the inside of John’s right ventricle, the pumping chamber to his lungs.
Lymphocyte infiltration, myocyte necrosis, endocardial fibrosis. Microscopic examination confirmed the cause of John’s heart failure. In plain terms, his heart muscle was inflamed, dying, and being replaced by scar tissue. His condition was most likely the result of a viral infection, a myocarditis. Scar cells don’t beat and are unable to pump.
After the procedure, John’s parents’ reaction when told of their son’s condition was predictable: “How do we treat this?”
They stood stunned and muted by the response: “A heart transplant.”
Just weeks before, John had been a normal high school sophomore unaware of any health problems, preparing to star in basketball. Now he was dying.
AT THE MOMENT OF his diagnosis, John and his family entered the dark side of medicine. In a twisted sense of the balance of life, for John to live, someone had to die. A viable, undamaged organ for transplant requires a quick strike to life, causing rapid brain death. The scenario almost always involves violence. The optimist views it as making the best of a horrible situation—saving one life instead of losing two. Still, the surgical team that harvests the organ, a team from the hospital that will implant the organ into another person’s chest, reminds me of grave robbers from centuries ago, who, with shovels in hand, looted cemeteries during the middle of the night by the light of oil lamps. Today, they are looting a body in a bright, clean, and sterile OR.
The downhill spiral of John’s failing heart was rapid. After his cardiac cath, he remained in the hospital and was placed on inotropic support—meaning that he was given intravenous medications to boost his heart’s ability to contract—while he awaited someone else’s misfortune. Fortunately for John, and unfortunately for some other poor soul, the progress toward his heart transplant was a sprint. Several weeks after his diagnosis, someone died suddenly, and that person’s heart came to beat beneath John’s breastbone.
During his heart transplant, looking over the ether screen into the chest cavity (with the paper drape, known among anesthesiologists as the “blood-brain barrier,” dividing the surgical area from my anesthesia nest), I saw that John’s heart wasn’t the size of a fist, but more like a melon, a cantaloupe; the firmness of a healthy muscle had given way to the bagginess of failure. And rather than bouncing, it was rocking from side to side, with the “la-lup” sound faint and indistinct.
After the transplant, which was successful, John received anesthesia on multiple occasions, including for subsequent heart caths for biopsies to measure his body’s attempt to reject his new heart. His initial recovery was remarkable for being unremarkable. My concern that anesthetics might hurt his heart lessened. He now contained a healthy heart.
Recovery for anyone receiving a heart transplant is a never-ending marathon. John’s new goal in life was to maintain the health of the heart in a foreign home, an unwilling and hostile environment that would rather not house it. He had to take multiple medications to stop his body from attacking the foreign heart muscle.
These drugs must be taken on schedule. Maintaining constant drug levels in the blood is imperative because the body’s defense mechanism for fighting invaders, in this case the foreign heart muscle, never rests. There are no medication holidays.
The standard seasonal illnesses that cause the common cold and the stomach flu become life threatening for the transplant patient if medications can’t be swallowed or held down. Hospitalizations are repeated and necessary to provide intravenous fluids and medications on a timely basis.
The longest-surviving transplanted heart to date has beat a little over a billion times, or thirty years. Eight out of ten heart transplant recipients survive the first year. Most will survive for twenty years, or 750 million heartbeats. That’s a very large number, but it is far less than three billion, the number of heartbeats in a normal life. So, the transplanted heart is not a guarantee into old age. It is a lease on life that will need to be renewed. This is especially true for young recipients. The transplanted heart gradually develops narrowed blood vessels—a vasculopathy—a perverse injustice resulting from the same medications that prevent organ rejection. Heart failure eventually recurs.
John’s parents dedicated themselves to his welfare. His medications were always available, his schedule maintained, his checkups routine, all recommendations followed. For teenagers, this rigid schedule is difficult. It’s a confinement that ostracizes these kids in times of rapid growth—physically, psychologically, and socially. School trips, outings with friends, late-night parties, and vacations all harbor threats. Drinking binges and hangovers are forbidden. Life is never as it was, or should be.
But John was committed to the regimen, and a couple of years later, he outgrew our pediatric hospital and his care was transferred to adult medicine.
AROUND THE SAME TIME, eleven-year-old Bandul came to us with his heart in worse condition than John’s. The son of undocumented immigrants, he was also uninsured. Few countries in the world would allow Bandul, unable to cover his costs, to enter an ER, let alone provide all necessary care until complete resolution of his heart disease.
With a bucket of unexpected surplus revenue, a bonus from an uptick in the economy, the state legislature had opted to pass the good fortune on to sick and needy kids. A Medicaid plan (state-managed care for the poor), increasing funds for children of families unable to afford health care—regardless of citizenship or even immigrant status—found receptive legislators not worried about raising taxes and losing votes. Bandul benefited from this new plan and received the same quality and quantity of care as John, no questions asked. At least from my anesthesia perspective, the care for Bandul and John was identical.
But after Bandul’s cardiac cath and diagnosis, he navigated a more ominous course than John’s. Bandul’s heart was failing more dramatically, and it worsened during the wait for that unknown someone special to die. He required a procedure attaching him to an external pump that assisted his heart’s contractions and blood flow—a ventricular assist device (VAD). Not only was his anesthesia complex, but just moving him from the ICU to the OR was a monumental task filled with potential risk. Luck shone on Bandul, though, as he, too, received another unfortunate’s heart.
After the transplant, Bandul remained in the hospital longer than John did. His native heart had been unable to support his kidneys, causing damage that took longer to heal after his heart transplant. But Bandul was eventually discharged on the same medications, and with the same needs, as John.
Great lengths were taken to ensure that Bandul’s non-English-speaking family understood the need for the medicines to be taken on a timely basis, for return visits to clinics not be missed. (On any given day, twenty percent of Medicaid outpatient appointments are missed.) Any variation from the rigid schedule could injure Bandul’s new heart.
Bandul’s parents were diligent, and he returned as planned for all of his appointments. A strong support group was built around him. As Bandul grew, the tone of the physician notes changed. At eighteen, Bandul was charged with the responsibility of taking his medications unprompted and of scheduling his own appointments. Bandul’s chart indicated that on questioning, he knew all his medications by name, could list the times for taking all the pills, and knew the phone numbers for his doctors, clinic, and hospital by heart.
But death always lurks near heart transplant patients.
I met Bandul again when he was midway through his twentieth year. The night before, he’d been admitted through the ER with complaints of abdominal pain and diarrhea. He needed a plastic tube threaded through a vein in his arm t
o his replaced heart to administer his medications and obtain blood samples. Bandul’s body was rejecting his transplanted heart.
He appeared healthy—thin but robust. He was buff, with firm biceps and leg muscles. And he was polite, readily answering my questions.
“Why did you get that tattoo?” I asked, referring to the giant tiger tattoo overlying his biceps on one arm. That tattoo in a patient with a transplant indicated risky behavior and rash decisions.
“It’s for kickboxing.”
“What?”
“It represents fighters and kickboxing.”
“You kickbox? You’ve had a heart transplant.”
“The doctors said I could. They said it was all right if I was careful.”
Bandul’s anesthesia was uneventful. His heart rate and blood pressure formed railroad tracks on the anesthesia record, the straight and flat lines of X’s and dots that I crave. After the procedure, and without apparent harm, I brought Bandul back to the intensive care unit.
But Bandul returned the following week because fluid was building up around his lungs, in his chest cavity—a pleural effusion. A tube to drain the fluid was needed. Bandul was in a torrid downward spiral. In medical lingo, he was circling the drain.
Another colleague was scheduled for the case, but because Bandul’s condition was tenuous, a pediatric cardiac anesthesiologist was required, so I met Bandul once more. I again reviewed his chart prior to the procedure, and the issues associated with his hospitalization were recorded more completely.
My own heart once again felt like a lead weight was hooked to it, pulling it down and out of my chest and into my belly.
Counting Backwards Page 8