Katy had both her lenses surgically removed when she was four. They were opacified by cataracts, the result of lifelong dependency on anti-inflammatory steroids. Afterwards, she required glasses about 3/8 of an inch thick. Eyes seen through that kind of correction fill the frames in a moony caricature – the professorial buffoon, the comic tyrant. To Katy’s startling owlishness, add puffy, rubrous cheeks, sparse, brittle white hair, mottled skin and a blocky, stunted frame. All of these features were animated by a stern litany of growls, unintelligible directives which she underscored using a jealously guarded laser pointer. Even without this bit of assistive technology, Katy had no trouble getting her point across, stabbing the air and roaring until the scene rearranged itself to her liking. She was never without her crimson, fur-trimmed Santa hat, even in the bathtub. It was impossible to resist a rueful grin around her, although whatever mirth she generated was tempered by an awareness of how close to death she lived every day of her life.
Katy was one of triplets, the result of infertility treatments. When they were born, her mother was 48 and her father 62. I was Katy’s pediatrician. Above my office desk, I keep a row of fat binders in which I’ve archived several thousand photos from more than three decades in practice. Early on in my professional past, these were taken by proud mothers using point and shoot cameras during exams. Later, most were selfies shot on smart phones, often wrested away from older sibs.
There are school photos, official and unofficial, celebrating the orderly progression of intellect and social adaptation – smiling young beings, gap-toothed, collars buttoned, hair immaculately combed or braided, aglow with health and unreserved delight. Many photos had arrived as holiday cards reflecting a year’s progress; children at birthday parties ripping into huge boxes, paddling happily in float rings around back yard pools, grinning children amidst their grinning families on exotic vacations, staged in group hugs at the beach, clasping boogie boards and snorkel gear, mom and dad festooned in flower leis and bold silkies.
Katy never lived any semblance of these lives. She is among the select few I have flagged in the many pages of my collection, those who died in childhood.
She appeared syndromic at birth, in dramatic contrast to her lithe, toe-headed, fine-featured siblings. Katy’s medical issues began in infancy with recurrent, mysterious symptoms – generalized nodular rashes, often blistered or pustular, clearly itchy or painful, swollen joints and fever. At intervals of weeks to months, she would experience a few days of misery, receive improvised comfort measures, and resume her unbothered existence.
Her milestones were delayed. She managed to sit independently at a year of age, walking at two. Katy remained non-verbal well into toddlerhood. It was also increasingly clear that her vision was markedly impaired. She sat inches from the TV, squinting and tilting her head at the screen.
I collected many photos of Katy. Typically, she is mooning at the camera between her beaming siblings, never without a Santa hat, its white fur border pulled down to the rim of her glasses.
Katy’s sibs, Anne and David, remained paragons of health and beauty all of her life. Absorbed in school, play dates, soccer and swim matches, lost in the blessed oblivion of childhood, they seemed to somehow escape the ravening black hole of Katy’s needs.
There was also older brother, Cal, whose life in between infrequent clinic visits I knew little about, He occasionally attended Katy’s clinic visits as a bystander. In the exam room, Cal would look up now and then from his homework or a hand-held game, and I’d glimpse in his eyes a note of apprehension, not so much worry for Katy, perhaps, as for himself.
Charming as she was, and solicitous as her siblings may have been, Katy’s illness was like a captive tiger that could turn on its keepers at any time, ripping the whole family apart. There could never have been more than a scrap of parental attention left over to nourish and succor her siblings, although whenever I saw them together, they never appeared as other than obliging and resourceful amusements for their odd sister.
Katy’s medical life was attended by an ever-evolving team of pediatric sub-specialists at various academic institutions in the San Francisco Bay Area, assembled according to her shifting array of symptoms. On the other hand, her medical “home” for lower level care was a community hospital in the East Bay where I practiced. All the many forays into the labyrinth of her symptomatology wound up in cul-de-sacs.
One memorable photo of Katy I enlarged and pinned up on a bulletin board in one of my exam rooms recalls a morning when I was making hospital rounds. She is sitting cross-legged in her favorite crib whose metal bars are enameled in lime green, clasping a remote TV speaker to her ear. Several attendants are fussing with the monitor and straightening the bedding around her. I remember her holding court as usual, barking orders in her husky growl and high-fiving everyone in the room. Her main point of focus would be Barney the Purple Dinosaur, her great favorite, grinning at us from the wall-mounted TV. Katy would be wagging a finger to the beat of Barney’s theme music with imperious precision.
A week before, she’d come close to dying from the first of many life-threatening GI hemorrhages, spending a week at a nearby pediatric intensive care unit. The bleeding had stopped on its own and, after a transfusion, Katy was able to return to her home facility.
I had met with her parents for an update down the hall in the playroom, leaving Katy happily caged in her crib, and, over bad coffee, somberly rehearsed the current array of threats to her life, which now included both GI and pulmonary circulatory disturbances, severe liver disease and a low platelet count which put her at risk of further serious bleeding. On this particular morning, I’d assembled an expanded team of medical participants for an in-depth, system-by-system stock-taking, including her nurse, a GI and pulmonary specialist, a nutritionist, and her social worker.
After we’d all settled into the cramped space, Katy herself joined us. We adults sat on kindergarten chairs pulled into a semicircle with Katy, cheery, pale Buddha in Santa attire, perched in front of us on her power quilt. As I trotted out a list of symptoms one at a time, Katy dialed away wildly on an Etch-A-Sketch with the intentness of a street fair portraitist, beaming at the results, holding up the screen to each of us as if it revealed the Ten Commandments.
This was actually the first time I’d spent an uninterrupted hour in her presence. Hearing her cackle with joy at each re-arrangement of lines, at the jingle of tin bells on her hat, watching her applaud herself in a mirror, I realized how charmed and momentously happy Katy was in the cozy space she’d made her own, versus the perilous tunnel of threats she navigated in our imaginations.
Two weeks after this conference, once again due to brisk GI bleeding, Katy needed emergency transport from our suburban pediatric ward to the pediatric unit at UCSF medical center. Although it seemed likely she would slip into shock, her family and I had hurriedly decided not to move her to a more intensive care setting, at least not at first, a plan it was hard to defend to the accepting pediatrician at UCSF. In making this triage decision, without admitting it, her mother and I were really acting on a much larger, although as yet unstated dilemma about what measures we might undertake should Katy’s condition further deteriorate. Just how heroic, to be understood as intrusive, would be the care her parents would authorize? It was a discussion bound to take place sooner or later, for which, no matter how well rehearsed in the imagination, no one can adequately prepare.
After my clinic day, I drove into the city to visit Katy. I was directed to a room where Katy and her mother were situated on the sort of narrow pull-out chair where her parents had spent countless nights hunkered, swarmed by monitor alerts and requisite nursing intrusions. Katy lay unconscious across her mother’s outstretched arm, her breathing ragged and erratic.
That morning, before moving her to UCSF, I’d conferred with a pediatric surgeon, debating an emergency splenectomy, a maneuver aimed at arresting the gastrointestinal bleeding by raising her platelet count. By then, her team of doctors had pumped a tanker-full of blood products into Katy’s circulation, trying to get ahead of her torrential blood loss, but were falling farther and farther behind. She had slipped into shock. Splenectomy would be an extremely high-risk undertaking.
It was clear to me Katy was fast sinking to where no conceivable intervention could rescue her, where shock would irreversibly injure her kidneys, heart, and brain. The pediatric nurse manager had assembled a late-afternoon care conference comprised of herself, a pediatric floor nurse from Katy’s home facility who had driven into the city after working an all-night shift, a social worker, a hospice nurse, newly-added to her care team, her parents and older brother, Cal, then about 11, and myself. Our talk fell under a pall of self-imposed restraint that felt like a drafted script for Katy’s eulogy. Katy would be accepted into pediatric hospice care. The social worker said nothing but nodded sadly for emphasis at key junctures in the conversation. After polling all concerned, Katy’s parents decided on supportive, as opposed to “heroic” measures, that is, oxygen and basic fluids, what we call “wind and water”, and pain management. It was remarkable Katy endured for the two hours the group spent conferring together. I felt sure I would receive the inevitable call sometime later that night.
But instead, the next morning I learned that, a few hours after I and a stream of well-wishers had departed the serene tableau of her dying, Katy opened her eyes, sat up in bed and, crowing with delight, began to toss water at her stunned visitors. To those of us having conceded the fight, nothing was more typical, and unnerving, than rallies of this kind.
* * *
Katy, the medical conundrum, was the subject of ten volumes of medical records, replete with countless lab tests, biopsy reports and dozens more concerning invasive diagnostic procedures, hundreds of “progress” notes and consultations written in the lifeless argot of the profession. Beneath the featureless surface of her medical record, there roiled a great mystery.
In her brief life, Katy made more than a dozen visits to UCSF. Her insurance even covered three full days of evaluation at the NIH. The best pediatric thinking failed to comprehend her condition. She was like a blackboard scrawled with impenetrable algebraic figurations. Our hope was an intuitive flash, a sudden incisive shift in perspective that would reduce her to simple terms and yield a workable solution.
Lacking a unified diagnosis, I always felt her prognosis was grave. Katy spent whole seasons in intensive care. After more than 30 years in pediatric practice, I still consider Katy’s survival to age eight a miracle.
In the last six months of her life, Katy spent just two weeks at home. Hope, like the transfused cells in her circulation, was always short-lived. Besides GI and pulmonary bleeding, other major medical issues boiled over unpredictably but with regularity – worsening arthritis, painful rashes, fever and suspected though usually unproven infection, progressive liver disease, diarrhea and poor nutrition.
There were less acute problems that constantly got parked on a back burner: her progressive visual impairment, profound deafness, and lack of speech. Katy had hearing aides but usually refused to wear them. To communicate, she preferred self-invented signs, tactical gesticulations, various grunts and emphatic barks, and, of course, her laser pointer. As for her poor vision, Katy kept dislodging the ‘permanent’ contact lenses inserted after her cataract surgery until her ophthalmologist got tired of twice weekly visits to replace them, and left them out, leaving her strapped into her owlish goggles. These she seemed to happily accept, more as a fashion statement when admired in her pocket mirror, another constant accessory.
Katy hated to eat. She was, in medical terms, orally averse. Throughout her life, Katy never took much of anything substantial by mouth, seemed to be repelled by textures. Unable to sort out chewing and swallowing, she often ruminated mouthfuls of food for hours, eventually gagging and spitting them out. She finally received virtually all of her nutrition via a constant infusion through a gastric button inserted in her abdominal wall.
We presumed the root cause of her ills was auto-immune, a term that suggests far more than it explains, meaning some fundamental mistake in her body’s surveillance system against foreign invasion in which many of its own cell lines had been accidentally targeted.
Compounding the mystery of Katy’s hyperactive immune system, was the contradictory fact that she was never able to mount an effective immune response to opportunistic infections, that is, make appreciable amounts of antibody to invading pathogens on her own. Although no one was certain of their benefit, she required monthly infusions of human immunoglobulin, a blood product comprised of broad spectrum antibodies, to maintain her at a level considered adequate to keep infections at bay. Minor infectious illnesses, such as simple colds or viral diarrhea, almost always put her back in the hospital, frequently in intensive care.
Naturally, Katy’s mother was prone to periods of despair, with brief remissions, even erratic spikes of optimism. Often, when Katy’s rush to the vortex was especially headstrong, her mother was tempted to abandon the powerful urge to toss her yet another life ring, and to let Katy go. In this regard, she was often encouraged by well meant, but, in my view, overly pessimistic advice from Katy’s medical care team, whether local or at an academic referral center, and by friends and family.
There were times when, bleak as Katy’s prospects seemed, I felt that to deny her certain interventions, even those which might be called heroic, was premature, that she could be rescued, if temporarily, restored to the meta-stable circumstances in which she so brilliantly thrived. I never could forget that epic emergence from coma to engage in yet one more water fight. We could never confidently know just how late it was in the game.
In emergent circumstances, the typical dilemma now was whether or not to return her to an academic referral center, relinquishing control over medical management, inevitably subjecting her to more testing and painful procedures, or to let her die near or at home. Her final stay at UCSF illustrated this quandary.
Katy was in our local ER once more, bleeding not just from her GI tract, but also her lungs, coughing up copious volumes of blood, in fact at immediate risk of drowning.
If we sent her back to UCSF, it would be in order for a pediatric pulmonologist to perform bronchoscopy under very adverse circumstances, passing a tube into her trachea to look for and hopefully stanch the source of bleeding. Bronchoscopy, which would require general anesthesia, would leave her reliant on a mechanical ventilator, at least during recovery, but possibly permanently.
In this scenario, as I explained to her mother, it was possible Katy’s family would be faced with the choice of whether to actively withdraw respiratory support, to “turn her off,” possibly in a fully sentient, if heavily sedated state. Quite reasonably, the pulmonologist at UCSF, who contributed his opinion via phone conference, as well as her local pediatric care team, were very discouraging.
In spite of the odds, there appeared to me to be one or two narrow, though perilous, escape ways back to some kind of safe haven. Initially, Katy’s mother opposed transfer and further invasive procedures, wishing only to provide comfort, “wind and water,” making sure Katy received sufficient morphine to endure drowning in her own blood. This would almost certainly require the induction of a comatose state, which itself might arrest her breathing. There was considerable local apprehension about this scenario playing itself out on a short-stay community pediatric ward like our own.
In spite of justifiable pessimism, we were all reminded of Katy’s many “miraculous” recoveries from perilous circumstances. I argued in favor of transfer to an intensive care setting, to include bronchoscopy, if that’s what it would take to stop the bleeding. Even if she required mechanical ventilation, her lungs were fundamentally sound and, once blood was cleared from her airways, I thought there was a good chance it would be temporary. Further, I thought we should take advantage of Katy’s time under anesthesia to perform colonoscopy, looking for potential sources of GI bleeding, which might be cauterized or tied off.
We decided to move her to UCSF. It was a stormy first day in the PICU, where Katy received more transfusions and an attempt was made at so-called non-invasive assisted ventilation, basically a mask tightly strapped to her face pushing oxygen under pressure into her lungs to try to damper the bleeding. Katy tolerated this poorly. Watching her struggle, Katy’s mother, having allowed her transfer, vacillated about bronchoscopy.
I had reviewed Katy’s admission history at UCSF, which was sent to me as a courtesy. The details of her story were complete and balanced, but I found the final summation of her care plan grating. Each note ended with “Katy is DNR,” “Do Not Resuscitate”. “DNR” status meant there would be no Code Blue called, that no specially skilled team would be summoned in the event of a cardiopulmonary arrest or other life-threatening situation.
I felt that DNR was an expedient over-reaction to Katy’s circumstances, dire as they might seem. I knew the mind-set that “DNR” status generates in physicians, especially those in training. It is the first and often the only guidepost they will fix on during a crisis.
Even given detailed contingency plans, physicians must never be excused from having to think, to respond to adverse circumstances with creativity and flexibility. They must accept responsibility for risky improvisations in response to an unpredictable turn of events, rather than staying the predicted course. I have seen more than one physician who thought he was navigating in accord with a senior consultant’s directions agreed to on sign-out rounds sail into disaster during the night by refusing to change course in adverse weather. So long as he or she is motivated by the Hippocratic Oath, first to do no harm, and next the Golden Rule, a physician, even one unfamiliar with a complicated case like Katy’s, will generally make the right decision.
I decided to fight traffic after I’d made local hospital rounds and join the UCSF team for their evening sign-out rounds. I had called Katy’s mother earlier in the day to advocate for a more nuanced plan after she had agreed to bronchoscopy and colonoscopy under one sedation. With this in mind, after protracted discussion, observing furtive eye rolling and sidelong glances among the residents and fellows assembled on rounds, I, the “local slick”, as residents at elite institutions referred to hometown providers in my day, prevailed on the UCSF team to consider reversing Katy’s DNR status.
The procedures in question were accomplished the following morning, and, remarkably as always, Katy tolerated both well. Fresh blood was found welling up from the bronchial tree, but without a discernable source. After it was washed out, there was no more active bleeding. The bronchoscope was successfully removed from Katy’s trachea without having to leave behind a breathing tube. Colonoscopy revealed numerous distended, thin-walled varicosities in the lower GI tract, reflecting circulatory pressure build up due to her advanced liver disease, none, however, actively bleeding. It looked like Katy would live to see another hospital discharge.
But then, of course, in just a matter of months, there was the inevitable hospitalization from which there would be no discharge. Katy had bounced in and out of hospice several times in the interval. Her parents had decided she had made her last trip to the PICU.
There was a somber gathering in Katy’s room, this time including a chaplain. Seated in a shadowy corner was a pediatric unit assistant who had become so close to the family as to fill in at home when one of Katy’s nannies was unavailable. She pressed a handkerchief to her face.
Katy had lapsed into stupor. On a CT scan, it appeared she had suffered a series of hemorrhagic strokes, entirely expected in view of her marginal blood clotting capacity. All kept silent during my system-by-system recitation of perils, almost all with eyes on the floor. I stated clearly that I felt no further interventions were warranted, that Katy should receive just comfort care from now on, and that it was time for her to return home if that’s what her family desired, for her final days among us.
When I finished the short litany of morbid scenarios, there was a further interval of silence, during which the regular beeping of hallway monitors and the whirr of a microwave oven in the kitchen nook next door enhanced the feeling of disproportion between Katy’s morbid entrapment and the airiness of ordinary life. Katy’s brother, Cal, finally spoke up, saying words close to those I myself had once spoken, citing her miraculous rallies, offering a modest, minority view in support of at least some respiratory assistance. This was met with respectful nods and a few more moments of silence. Her mother then asked me to speak at Katy’s funeral.
I have lived this day many times in the last eight years. But with Katy’s death, the sadness I have felt in its anticipation has been transformed into something else. Looking at your glowing faces, I have a strong sense that this transformation has occurred in you as well.
Surely we all knew that Katy was marked nearly from the moment of her birth. But for what distinction? I think we all assumed for tragic suffering. For years, we probed the conundrum of her symptoms, improvised her care without the remotest knowledge of what we were treating. As we struggled for a plan, Katy hovered at the brink. We could only watch in amazement as she danced away from death, time and time again, in defiance of every scientific principle, beckoning us to follow as she scampered into the next green mansion in the magic kingdom that was her native habitat. Katy turned science on its head, where it mocked us like a grinning Cheshire cat.
Many of us were privileged to get close enough now and then to observe Katy in her native element and there we could no longer feel so bereft, that her life was in any way a tragedy. I rather came to think of it as a triumph.
Who can imagine a Katy other than the ebullient being in a pulled down Santa hat, roaring approval at Barney? A cautionary finger lifted, she silenced us all, scribbling wildly on her Etch-a-Sketch, as if taking dictation from a higher authority, calling on each of us to applaud her latest attainment. “Pay attention,” was Katy’s message to us all, “to what sounds like nonsense and looks like gibberish and consider your need for certainty and progress as you try to make sense of the world. I’ve got it figured out. Just watch.”
Her world made perfect sense to Katy and her brief time in ours has left me feeling as if much of my striving to understand life in scientific terms, to insist on certainty before advancing into the unknown, is squandered time. That was Katy’s wisdom and enduring gift.
Stevan Cavalier is a retired physician, writer, artist, photographer and professional pianist. BA (cum laude) English Amherst College. American Academy of Poet’s Prize.