Real Life Impacts: The Story of Stephanie Smith
Stephanie Smith—Before Her E. coli O157:H7 infection
Stephanie Smith was born on February 20, 1987 in St. Cloud, Minnesota, and was something of a miracle from the start. He mother, Sharon, was pregnant with twins when, at eight months, sudden hemorrhaging revealed an ectopic pregnancy. She recalls: “Stephanie’s sibling stayed in the fallopian tubes, but Stephanie had moved to the uterus. She was the stubborn one, starting at conception.”
The family moved to Cold Spring, where the children did most of their growing up. Her brother, Will, remembers that they liked their new schools, and both fit right in. He also remembers this being the time period that Stephanie started dancing. “Mom was working lots, and I was in after school activities, so a lot of times I ended up going to her practices. She stood out from the rest of the dancers.”
Just like with her brother, Stephanie enjoyed an unwaveringly strong relationship with her mother. “We were always bonded,” Sharon says, while Stephanie remarks, more effusively, that Sharon “has always been the best mom I could ever ask for.”
Stephanie grew quickly into an independent woman. At age fourteen, she began working. Then at 17, she began to teach dance with a regional organization called Just for Kix. And by age eighteen, she had taken significant steps toward opening a Just for Kix location in Paynesville, Minnesota, a location that continues to be highly successful. Stephanie worked daily to set up the program, including incorporating the business, working through insurance issues, and selecting the location, which remains the same today. And as if this was not enough for a high-school senior, Stephanie also helped run a business out of her home called “Upper Case Living,” and began a dance line at her alma mater, Rocori High School, called the Rocori Rockettes. She had truly become a confident, vibrant young woman.
Even the briefest of overviews of Stephanie’s pre-illness life would be incomplete without a mention of dance. For Stephanie, dance was not merely a passion—more than that, it was a driving force in her life. She loved to dance; she loved to teach dance; and she had every intention of making dance her life’s work. Just as she had already started the Paynesville location of Just for Kix, she planned to open a studio in Arizona too, at least when she finally moved there. Sadly though, this was never to be.
Stephanie’s Devastating E. coli Infection
Onset of E. coli Illness, And Then a Rapid Decline
On September 7, 2007, Stephanie’s aunts bought a box of American Chef’s Selection Angus Beef Patties from a Sam’s Club. They brought the box of patties back to Sharon’s house, and placed them in her freezer to be ready for a family barbecue planned for September 22. At the barbecue, Stephanie ate at least one hamburger made from the patties, and noticed nothing wrong.
On Thursday, September 27, she developed a fever, and had diarrhea and chills. She was not overly concerned though. That changed when, the next day, she suffered multiple bouts of increasingly severe bloody diarrhea. This prompted her to go to the Emergency Department at St. Cloud Hospital. There, the attending physician suspected an E. coli O157:H7 infection and so ordered that a stool sample be tested for E. coli bacteria. Meanwhile, Stephanie was admitted to the hospital for treatment with intravenous fluids and morphine for her mounting pain.
Despite feeling fine just days before, Stephanie’s health began a profound decline. Over the following days the severe damage being caused to Stephanie extended to her brain. Early on the morning of October 2, a lab technician drawing blood noticed some unusual spastic movements in Stephanie’s arms and legs. A nurse was summoned, and she observed Stephanie staring vacantly into space, and unable to speak in complete sentences. A doctor arrived within fifteen minutes, by which point Stephanie’s body had begun to shake and jerk. Her speech had also become slurred, which made it difficult to understand Stephanie’s answers to his questions. As her condition continued to worsen, Stephanie was transferred to the Intensive Care Unit (ICU) where the onset of kidney failure was detected.
The ICU attending physician (or intensivist) who had by now assumed responsibility for Stephanie’s care, soon recognized the significance of the three primary diagnoses—hemolytic anemia, thrombocytopenia, and acute renal failure. This triad of conditions represented a confirmation of the onset of hemolytic uremic syndrome (HUS), a rare illness that is diagnosed in less than 10% of E. coli patients. The doctor therefore discontinued antibiotic therapy, except Invanz, and requested neurology, nephrology and gastroenterology consultations.
A nephrologist was the first specialist to examine Stephanie. Although awake, she was unable to respond appropriately to questioning. The doctor noted a generalized edema (swelling)—in fact, Stephanie had gained nearly fifteen pounds since admission to the hospital. This edema was in large part the result of reduced urine production—another sign of kidney failure. With the grave nature of her condition readily apparent, Stephanie was prepared for dialysis and plasma exchange.
Stephanie remained lethargic and semi-responsive, varying between confused and disoriented. The seizure activity also continued without stop, her arms and legs shaking, and her eyes jerking and unable to track together. A neurologist performed a bedside EEG which revealed a general slowing in brain function. A subsequent MRI of the brain was also normal. This left the doctor to treat the ongoing seizures with anti-convulsant therapy, including the administration of multiple drugs.
Overnight, and throughout the following day, Stephanie’s condition continued to worsen. She was now oliguric—that is, producing less than 500 cc of urine in a 24-hour period. In addition, her worsening neurological status was beginning to interfere with her ability to breathe on her own. As a result, Stephanie had to be sedated, and then intubated, and placed on mechanical ventilation.
Twenty-four hours later, on October 4, Stephanie stopped producing urine, a sign of complete kidney failure. As a result, she needed to be started on hemodialysis, which meant another machine doing the work once done by her own body. She would remain on dialysis for weeks.
As for the neurologic condition of her brain, things looked increasingly bleak. On the same day that she started on dialysis, she scored a 10 on the Glasgow Coma Scale. The next two days also brought continued seizure activity that prevented Stephanie from meaningful cooperation or response of any kind. And, on October 6, she suffered a grand mal seizure that lasted for over an hour, despite repeated administration of anticonvulsant medications.
After the grand mal seizure, a neurologist advised Sharon that seizures as persistent and strong as Stephanie’s were commonly associated with incomplete recoveries. Further, the anti-convulsants that her daughter was currently on only suppressed the seizure activity by about ten percent. Doctors thus believed that Stephanie needed to be placed in a drug-induced coma with continuous EEG monitoring. Sharon gave tearful consent, and Stephanie was loaded with medications in an effort to finally end the relentless seizures.
At this point, as Stephanie’s body appeared to calm, Sharon hoped beyond hope that her daughter had found some peace at last. She could not help to fear, however, that such stillness might be an awful omen, and that there might not now be any end to coma. That is why when the doctors told her that Stephanie would be transferred to St. Mary’s Hospital, part of the Mayo Clinic, she immediately felt more optimistic.
Over Three Long Months of Pain, Fear, and Uncertainty
Prolonged Kidney Failure and Status Epilepticus—A Brain in a State of Acute, Persistent Seizure
On October 7, once she had stabilized, Stephanie was airlifted to St. Mary’s Hospital in Rochester, part of the Mayo Clinic. Her condition was critical on arrival, and she was directly admitted to the Neuroscience ICU. The immediate treatment plan included plasmapheresis and hemodialysis, and maintenance of the drug-induced coma. She was to remain on the mechanical ventilator, and was to receive nutrition from a percutaneous endoscopic gastrostomy (PEG) tube.
Over the course of the next two months, Stephanie would suffer from a variety of severe complications. Infections, of course, were also a big problem. During the her stay at St. Mary’s, Stephanie developed, among other things, pneumonia and multiple urinary tract infections.
Quite clearly, however, Stephanie’s primary problems during her hospitalization at Mayo Clinic remained HUS and status epilepticus. Her kidneys were non-functional, producing very little, if any, urine, from October 3 through October 24. During this time period, she received continuous renal replacement therapy (CRRT). She also received numerous blood transfusions to address continuing anemia and thrombocytopenia. When Stephanie finally did start to produce urine again, it was of such poor uremic quality that she had to continue to receive hemodialysis three to four times a week. This lasted until November 20.
Neurologically, Stephanie still could not breathe on her own, and she remained in a drug-induced coma during the months of October, November, and into December. Clinical seizure activity was often evident, but subclinical monitoring by continuous EEG showed seizures much more frequently, punctuated by “bursts” of epileptiform activity. Typically, these bursts followed any sort of stimulation, especially the dialysis procedures. The bursts following dialysis occurred, at least in part, because the anti-seizure drugs were, at that point, being scrubbed from her blood by the dialysis machine. Her many neurologists repeatedly adjusted her anticonvulsant therapy to try to stop the increased seizure activity during dialysis, but they could do little more than wait for kidney function to recover so that dialysis might no longer be needed, and a steady level of medication achieved.
Through all of this, Stephanie’s family stood vigil, hoping and praying for any sign that she might regain consciousness. But for well over a month, there was no sign at all. And then, on November 12, a neurologist who had long cared for her noticed that Stephanie was “awake” enough to be triggering the ventilator—in other words, she still had some independent respiratory drive. Then two days later, Stephanie was able to start on a form of respiratory therapy intended to wean her from the ventilator—something called Continuous Positive Airway Pressure, or CPAP. Although by no means breathing on her own, this was still a big improvement, and a sign of her potential for recovery.
By December 2, Stephanie began to open her eyes spontaneously. Though the initial progress was short-lived—seizures actually resumed afterward—it was progress nonetheless. If nothing else, it gave doctors, nurses, and family alike hope that Stephanie would make a recovery.
Imaging studies of Stephanie’s brain did not paint a bright picture. Her neurologists agreed that this all represented the effect of the hemolytic process that had been set into motion by her E. coli O157:H7 infection.
Discovering the Full Effects of Hypoxic Brain Injury
Over three months into her hospital stay, Stephanie was finally removed from mechanical ventilation at 1:00 AM on December 14. She was not, however, ready to breathe on her own. Instead, she was transitioned from the ventilator to supplemental oxygen that she received through her tracheostomy tube.
Much uncertainty was left, however, when it came to assessing Stephanie’s brain function. By now, her kidneys were functioning enough for her to no longer need dialysis, and so the focus of her medical care was changing accordingly. Continuous EEG monitoring showed no seizure activity, but there was also no evidence of visual fixation or tracking. As a result, the family was warned not to misinterpret the significance of Stephanie’s apparent ability to look around the room. In fact, doctors explicitly cautioned that Stephanie still could slip into a vegetative state as a result of her profound neurological injury.
And so, despite the fact that Stephanie truly had progressed, full answers to the question how extensive her brain damage was continued to be elusive. For the rest of the month, Stephanie demonstrated rudimentary neurological function:
- December 18: Stephanie smiled multiple times during the day.
December 20: Stephanie tracked the neurologist around the room, but was unable to follow the neurologist’s finger visually. December 21: Stephanie fixed, tracked, and was able to follow one simple command: smiling at her father when instructed to do so. December 22: Stephanie was able to fix and track more consistently, and followed the neurologist’s finger vertically and horizontally. December 24: EEG monitoring, which had been continuous since her admission to Mayo Clinic, was discontinued.December 31: Stephanie continued to track, but was unresponsive to painful stimuli and was unable to follow simple commands.
By the end of the year, Stephanie’s list of physical problems remained dauntingly long and complicated. As 2008 began, there seemed little to celebrate. The improvements that had once appeared momentous were now long-gone memories, replaced by the impossible-to-ignore reality of a Stephanie who no longer seemed to be there, except as a body. On one hand, with some recent signs of increased alertness, there was cause for hope. On the other hand, with her continuing, severe limitations, there was a need to be realistic about what to expect by way of possible recovery.
The time had thus arrived to discuss “placement options” for Stephanie. The most desirable option, in the family’s view, was for Stephanie to remain at the Mayo Clinic for inpatient rehabilitation to help her regain as much independence, and relearn as much function, as possible. But she had been provisionally accepted to the Assumption Home skilled facility, in the event that rehabilitation at Mayo Clinic was not possible. Sharon began to work with a social worker and insurance specialist to figure out possibilities.
It was by now clear that the primary barrier to rehabilitation, much less regaining any independence whatsoever, was profound psychomotor retardation—which is to say, a significant slowness of thought, limited capacity for functional communication, and the severely restricted ability to move. Nevertheless, Stephanie continued to make modest, incremental gains throughout the rest of her stay at Mayo Clinic’s hospital, including:
- January 9: speech and language therapy began with limited goals, including improving the ability to follow basic commands, give yes or no responses, or achieve any type of consistent functional communication.
January 14: physical and occupational therapy began, also with limited goals. January 16/17: she was progressively more alert, and was able to follow blink commands, tracked better, and could lift her arms from the bed. January 18: the neurologist noted that Stephanie was “the most awake and alert I have seen her”; upper extremity movements were purposeful and with more strength; she was also able to nod her head to yes or no questions, and even asked for her mother. January 24: the neurologist noted that Stephanie withdrew her lower extremities to painful stimuli.January 26: she ate pureed foods, orally, for the first time since being admitted to treatment.
The foregoing gains were evidence of slight improvement, but their very slightness spoke to just how long the road to even a minimal recovery truly was going to be.
Stephanie’s gradual awakening was equal parts miracle and curse. One can only speculate as to the devastating personal pain Stephanie endured upon realizing that she could hardly move. With a simplicity that certainly belied what must have been an all-consuming fear, Stephanie remarked on January 28, 2008, “I can’t move my legs.” For the former dancer, reality was beginning to set in.
On January 29, the day of her discharge to the Mayo Clinic Acute Inpatient rehabilitation, the medical records note that Stephanie had no sensation to pinprick in her legs, no spontaneous movement, and no movement to command below the waist.
The Unending Ordeal of Stephanie’s Rehabilitation
Mayo Clinic Acute Inpatient Rehabilitation: January 29 through February 26, 2008.
After being settled into the rehabilitation unit, Stephanie was evaluated by a physical medicine and brain rehabilitation specialist. His impression, overall, was that Stephanie was suffering from clear cognitive deficits, a profound lower extremity paralysis (plegia), generalized de-conditioning, and pathological crying. The rehabilitation plan formulated included: (1) continued physical therapy for upper extremity strengthening and increased mobility; (2) occupational therapy for neuromuscular re-education; and (3) speech therapy to continue in the effort to develop any form of functional communication possible. At the time of admission to inpatient rehabilitation, her only forms of communication were single words and simple gestures.
Stephanie’s neurological improvement over the course of her month-long stay at Mayo Clinic’s rehabilitation unit was consistent, but quite modest. On January 31, she tearfully participated in assembling a 12-piece puzzle. Over the next 3 weeks, her vocabulary and executive functioning steadily increased to the point that, by February 20, she was able to play a card game with a visiting doctor. Her memory, however, remained profoundly compromised. And, physically, she continued to have little sensation in her legs, and no ability to move them.
Overall, Stephanie’s general prognosis and outlook remained very poor. She was obviously grief-stricken, and was well aware that the profound injuries she had suffered had changed her life’s trajectory completely. By the end of her stay at Mayo’s rehabilitation unit, this formerly supremely healthy and active young woman well-knew that the odds of ever being able to walk were overwhelmingly poor.
Struggling to Get Better, Closer to Home—Adult Rehabilitation, February 27 through April 17, 2008
On admission to St. Cloud Hospital, rehabilitation specialists immediately assessed Stephanie in order to come up with their therapy plan. Aside from the obvious paraplegia, what they found was a severely depressed young woman with profound cognitive deficits, including extensive memory and recall problems, limited problem-solving capacity, and moderate to severe cognitive-linguistic deficits.
Stephanie’s associated physical limitations continued to be divided at the waist. Though her motor coordination continued to be impaired, her arms and trunk were certainly gaining strength, mobility, and general functionality. But sadly, it was by now clear that Stephanie’s inability to use her legs was not simply due to a transient, critical illness myopathy/polyneuropathy. Instead, her lack of progress during her already-long rehabilitative course suggested that Stephanie had sustained a motor-neuron injury that had irreversibly severed functional communication between her legs and brain.
Over the first several weeks at St. Cloud’s, Stephanie’s progress was punctuated by continuing bouts of severe depression, combativeness and general emotional instability. Despite treatment, Stephanie remained emotionally devastated about what had happened to her, and her bleak future. On March 3, she told one of her therapists that “I don’t want to get better because I can’t have babies.” Stephanie was ultimately diagnosed with involuntary emotional expressive disorder (IEED), but this was a name for her condition, not a cure.
Stephanie underwent daily therapies at St. Cloud Hospital during her stay. She continued to make progress with functionality in her upper body, generally, but demonstrated clear cognitive impairments. Significant events over the course of her stay at St. Cloud include:
- March 6: Stephanie was fast-achieving independence with basic activities of daily living, including being able to open a tube of toothpaste and brush her teeth with only moderate verbal cues and minimal physical assistance; on this date, Stephanie also completed a functional problem solving task with 100% accuracy.
March 10: Stephanie could state the days of the week backward and forward, and she knew the month and the year. March 11: Stephanie completed the Rivermead Short Term Memory Test, but scored only 2 out of 24 possible points, indicating a severe memory deficit impairment. March 12: Stephanie had begun participating in some rolling activity, had demonstrated improvement in her sitting balance, and had begun to propel her wheelchair. March 18: Stephanie immediately recalled five out of five items during a memory test, and also demonstrated sustained attention during her exercises with her cognitive therapist. March 19: Stephanie demonstrated continuing progress in sitting balance, rolling, and bed mobility, but was still “max dependent” for transfers from bed to her wheelchair. March 26: Stephanie again completed the Rivermead Short Term Memory Test, scoring 8 out of 24 possible points. Despite the improvement, Stephanie’s score still indicated a severe impairment.
By the middle of April Stephanie’s depression issues required her to be transferred to the Bethesda Hospital Adult Neurology Rehabilitation Program in St. Paul. The transfer occurred on April 17.
Neurology Rehabilitation, April 17—June 18, 2008
Although her environs had changed, sadly, Stephanie’s daily activities stayed the same. She received intense occupational, physical, and communication therapies, as well as psychiatric and behavior management to control her depression and impulsivity. Indeed, treating Stephanie’s severe depression continued to be of paramount importance. It was unanimously felt that her severe depression constituted the primary barrier to her reaching maximum improvement.
Doctors thought that, given Stephanie’s demoralization, depression, and general hopelessness, she would require psychiatric management and extensive psychotherapy on an ongoing basis, most probably always.
The progress achieved at Bethesda was, for the most part, only psychological. It was not as if much had changed except for a lessening of her depression and an improved outlook. Her legs remained functionally paralyzed. And despite being able to attend to a few basic self-cares, she still required the maximum assistance for all of her daily needs. Thus, when Stephanie was discharged home on June 18, 2008, the problems listed on her discharge-diagnosis were each as awful as they were overwhelming. These were:
- traumatic brain injury with reduced, though improving, cognitive function;
difficulty adjusting to the limitations of her medical condition; paraplegia; neurogenic bowel and bladder; and controlled seizure disorder.
Nearly a Year of Outpatient Rehabilitation Therapy—June 18, 2008 through April 26, 2009.
The evening of June 18, 2008, Stephanie finally returned home. Other than a few day-passes to visit with friends and relatives, it was the first time that she had been home since her admission to St. Cloud Hospital nearly ten months earlier. To make things more accessible, the house had undergone significant modifications. A ramp leading up to the front door had been built; the rear deck had been enlarged; and the dining room had been turned into Stephanie’s bedroom, which was formerly upstairs.
For the next eleven months, through April 2009, Stephanie lived at home. There, she continued to receive physical, occupational, and speech and language therapy. During this time, her permanent cognitive deficits and other neurological injuries, along with associated physical limitations, began to be better explained. For example, it was increasingly apparent that her limited organizational ability, and problems with executive functioning and memory, would prevent her from living on her own, or ever managing, in any meaningful manner, her own affairs. She certainly had little chance, if any, to be employed. In fact, in a letter that her doctor wrote to her insurance company dated January 2, 2009, it was explained that Stephanie was a “dependant incapable of self-support due to a brain injury, paralysis, and chronic medical conditions secondary to hemolytic uremic syndrome.”
Ultimately, the reality was that Stephanie’s severe neurological injury would prevent further progress. Stephanie could neither stand nor walk, and the doctor found no voluntary muscle activity, except for the right quadriceps and right anterior tibialis.
And as if all this was not enough, during this time period, Stephanie suffered from constantly high blood pressures. In fact, she had to be seen in the Emergency Department at St. Cloud Hospital, or had to be hospitalized, on at least two occasions for dangerously high blood pressure—e.g., blood pressures of 207/133 on November 1, 2008. Plainly, the high blood pressures were not good signs for the survival of her kidney function.
On March 19, 2009, a rehabilitation specialist visited Stephanie at home. After evaluating her, the specialist identified current problems as incontinence, transient lower-extremity pain, dizziness and nausea with standing, and cognitive and neurological deficits with associated difficulties completing activities of daily living. In summary, the specialist believed that Stephanie would be much better served by participating in an inpatient brain injury rehabilitation program, with specific, aggressive daily therapies.
Trying Inpatient Rehabilitation Again—April 27 through June 11, 2009
Stephanie ultimately agreed that she would be better off in an in-patient setting, at least insofar as it would give her another chance at more frequent, aggressive therapy. As a result, on April 27, she was admitted to a neurological therapy facility located in Bakersfield, California. Upon admission, long-term treatment goals included brain injury re-education through daily therapy, with a chief objective being to maximize Stephanie’s independence.
Stephanie enjoyed the environment much more than any facility she had been in to-date. She made a good effort to participate, and her therapy achieved modest successes. Nevertheless, with each passing day, she saw little improvement in the one area that mattered most to her: she still could not walk. And even worse was that it was becoming increasingly apparent that she would never do so.
At the time of her discharge, on June 11, Stephanie had endured forty-two days of aggressive rehabilitation. She had improved in a few areas, including many involving her cognitive deficits. But such improvement was relative, and it was measured against a low baseline. Consequently, Stephanie remained dependent on others to do the many things associated with ordinary living. She also continued to show no functional mobility in her legs. Finally, even though the experience at this facility had been mostly positive, Stephanie remained deeply depressed and angry about her circumstances.
The Medical Consequences of Stephanie Smith’s HUS Illness
Stephanie’s neurological injuries
Status Epilepticus. One of the recognized neurological complications of HUS is seizure activity. In most cases, particularly where the neurological-impact is associated with metabolic derangement, the seizures resolve without causing long-term sequelae. But Stephanie Smith experienced something very different from the sort of seizure-activity commonly seen in HUS. The neurological component of her HUS-illness expressed itself in the most pathologically severe manner possible: a self-sustaining neurological event described as “status epilepticus (extended seizures),” which doctors managed to control only by placing her in a long-lasting, induced coma.
There are at least two critical consequences of extended seizures: they become self-perpetuating, and they result in neuronal death. Status epilepticus also induces systemic changes with significant, immediate health risks.
The cause of Stephanie Smith’s cognitive deficits and spastic paraplegia. As will be discussed in greater detail below, Stephanie has undergone an array of neuropsychological tests that consistently document extensive cognitive deficits. The pathogenesis of such deficits is widespread white matter atrophy, which has been well-documented in the wake of status epilepticus. Brain MRI scans during Stephanie’s various hospitalizations confirm that she suffered white matter atrophy. In addition to the white matter abnormalities, “structural findings consistent with brain injury are also present, in the form of generalized atrophy as well as gray matter involvement, both in cortex and deep gray nuclei.”
The direct cause of Stephanie’s paraplegia is an extensive injury to motor neurons that occurred during, and as a result of, her extensive seizure-activity, and the thrombotic micro-vessel occlusion in her brain.
The lasting effects of Stephanie’s neurological injury.
Psychological injury - Stephanie has undergone rigorous neuropsychological testing and evaluation on several occasions from 2008-2010. Despite extensive psychotherapy, with strong pharmacological support, all evaluations revealed a severely depressed young woman, without happiness or hope, and no remaining capacity for inspiration.
Cognitive deficits - Stephanie suffers from myriad cognitive deficits as a result of her neurological injury. These have been variously described by several neuropsychologists, all of whom conclude that the deficits are profound and permanent.
Neurogenic bowel and bladder and spastic paraplegia - Stephanie’s motor neuron injury has manifested in essentially two ways: first, she does not feel significant urinary urgency and is functionally incontinent; and second, she has lost all functional control of her legs.
Sadly, both conditions are permanent. And with respect to ambulation, Stephanie’s continued rehabilitative efforts are merely intended to help her gain strength and mobility, primarily with respect to transfers (e.g. from bed to her wheelchair, or from wheelchair to the commode).
Stephanie’s renal prognosis is poor. She has been maintained on antihypertensive medications—currently amlodipine and metoprolol—since her acute hospitalization. Several leading nephrologists agree that Stephanie is headed for end stage renal disease. One expert estimates Stephanie’s risk for later renal complications including the need for dialysis and/or transplantation as greater than 90%.
Medical problems associated with End Stage Renal Disease
The kidneys do much more than simply cleanse the blood. As a result, the development of end stage renal disease is associated with a laundry list of often nightmarish medical problems. These may include:
- alterations in calcium and phosphate balance, known as renal osteodystrophy, that cause the bones to become weak and soft, resulting in bone pain and susceptibility to fracture;
anemia—which is characterized by a low red blood cell count and consequent lack of energy; in children, growth failure caused by the damaged kidney’s inability to properly regulate the calcium necessary for bone growth; and high blood pressure, which, among other things, stresses the heart and can lead to coronary artery disease, heart attack, and stroke.
Development of End Stage Renal Disease (ESRD) means the patient will require dialysis or transplantation for survival. Most Americans who suffer ESRD opt for a kidney transplant, but the wait for a donor kidney is often a year or more. If Stephanie does not have a living related donor, she will need peritoneal or hemodialysis until a donor is found. Once the ESRD patient has only about 15% of normal filtration capacity, they will start to need dialysis.
Following transplantation, Stephanie will require immunosuppressive medications for the rest of her life to prevent rejection of the transplanted kidney. Medications used to prevent rejection have considerable side effects.
Finally, because no kidney transplant lasts forever, Stephanie will face multiple transplants over the course of her lifetime. Each transplant will be preceded by ESRD, dialysis, an increase in kidney-related medical problems and then the recovery from transplantation.
From a purely physiological standpoint, the reality is that Stephanie should not become pregnant. The risks for both her and the fetus would be too extreme. Stephanie would be at “very high risk for toxemia (pre-eclampsia and eclampsia) during any pregnancy. Indeed, a long-term study has shown that women who survive HUS are at a roughly ten times higher incidence of toxemia than other women.
The potential consequences of becoming eclamptic include, for Stephanie, death or rapid acceleration of her kidney disease; and for the fetus, they would include premature birth and the life-altering problems that accompany them, such as epilepsy, cerebral palsy, and mental retardation, to name only a few.
Stephanie’s Medical Expenses
Medical expenses occurred over the first years of Stephanie’s E. coli infection, HUS and rehabilitation, totaled $1,974,871. Expert estimates for the cost of all aspects of Stephanie’s future medical needs totaled nearly $30 million in 2010 dollars.
New York Times journalist Michael Moss won a Pulitzer Prize for the telling of Stephanie's story in his article, "The Burger That Shattered Her Life."