Anonymous
APRIL 1993, CHILDREN'S HOSPITAL, BOSTON.
Our 12-year-old daughter Hillary had just been diagnosed with lupus nephritis. We were absolutely thunderstruck. We had never heard of lupus before, never heard of autoimmune diseases or glomerulonephritis. How could this be happening to our daughter, a competitive gymnast in prime physical condition, with not even a hint of ill health until just a few days prior?
HALLWAY OF "9 EAST" WING, CHILDREN'S HOSPITAL.
Hillary's kidneys were failing rapidly, we learned from the doctor. The nephrologist explained the life threatening risks at hand, sending our hearts pounding and the veins in our temples pulsing. She recommended beginning treatment protocols immediately, and in rapid fire, she introduced us to prednisone...nifedipine...cytoxan...blood transfusions...hemodialysis..., all strange and scary words to us. This was not a discussion, not a dialogue; it was a one-way communication to which we had no response. We were mentally flaming out. That night, we resolved to find out as much about lupus as swiftly as we could, not only to bring light to our ignorance, but to better understand the doctors' recommendations, to engage in intelligent dialogue with them, hopefully to enter into a collaborative partnership with them in seeking solutions for Hillary's condition.
3 A.M. There was no one we could reach at this hour of day in Boston, nor even on the West Coast; but it was mid-afternoon on the other side of the world. We proceeded to telephone some doctor friends in Perth, Australia, who quickly pointed us toward some useful information sources on lupus. We called relatives and medical friends in Sapporo, Japan, who promptly swung into action researching the medications. We contacted a very close family friend in Kuala Lumpur, Malaysia, who introduced us to doctors at the SLE Clinic at University Hospital. By late morning, we had spoken with a dozen or so people, and a rough picture of what lupus was all about began to form in our minds. We were encouraged, and we felt that Hillary's doctors would be pleased that we had "done our homework," That wasn't to be the case.
SNOWJOB. The doctors at Children's Hospital seemed put off by our desire to engage in dialogue. In so many words, they told us that we could not possibly understand all of what they knew (which was, of course, very true), and that "a little knowledges is worse than no knowledges" (which was absolutely false). One particular physician began to snow us with highly technical research papers on cyclophosphamides and corticosteroids. When we realized what was happening, we stepped up to the challenge.
We quickly realized that our most pressing need was to understand all the technical laboratory test jargon that the doctors were throwing at us, because those tests formed the basis upon which they were recommending treatment protocols. It wasn't that we didn't believe what the doctors were telling us in highly edited lay language; rather, as parents of a very sick child, we wanted to fully understand and participate in the decisions that would influence her treatment.
Fortunately, the most immediately available information source was right down the hall from
us - the reference books at the nurses' station, so that's where I spend the next several hours, reading about serum complement (Hillary's C3 and C4 had fallen through the floor), urine protein (normally 0-8 mg/dl, but hers was in the 4 digits), serum creatinine and BUN (both 2-3 times normal), ANA (hers was negative), and numerous other tests for which the hospital was drawing blood specimens every 12 hours. It was hard going for lay people such as my wife and me, but the nurses were especially supportive, suggesting alternative reference sources as our questions became more focused. We cornered the doctors-on-call as well as the floor nurses whenever we could to further elucidate the lab readings for us. We networked amongst relatives, neighbors, and friends to seek information from outside doctors, to double-check and triple-check our understanding of the rapidly mounting lab findings. Stage by stage, the fog lifted, and Hillary's doctors didn't sound so godlike anymore. Our medical knowledge was still a fraction of what the doctors knew, but we began to understand what we needed to understand. And then we discovered the hospital's computerized laboratory database.
ENABLING INFORMATION. As databases go, Children's Hospital's was rather crude, lacking any computational or graphical capabilities, but all the lab information was there! A sympathetic nurse kindly punched up the records on our daughter and gave us a printout of all the critical test results from the past several days and, scanning the trends, we immediately gained a whole new perspective of the battle that Hillary was bravely waging.... It was the doctors' perspective, and all of a sudden, the right questions began to emerge from our cluttered minds.
From that point on, we made it a point each day to keep lab records in parallel with the hospital's own, initially with pen and paper in a simple notebook, later by transcribing onto a spreadsheet in my laptop computer, which I brought into the hospital every evening. This effort enabled us to interact with the physicians much more cooperatively. We were no longer being talked at; we were in consultative dialogue. And then we discovered Medline, the computerized, searchable database of the National Library of Medicine, containing some 7 million biomedical references to articles published in 4.000 international journals worldwide from 1966 to the present.
CLASSIFICATION IV. Reading about the side effects, potential adverse reactions, and contraindications of these drugs were heartwrenching, as any parent will know, but step-by-step we began to understand the difficult choices and tradeoffs that the doctors had to contend with, and our posture toward them became much more dispassionate. This was not an exact science; it was, at best, a "statistical best guess" at what was optimal for the patient at hand. According to our daughter's nephrologist, an SLE patient falls into a particular classification based on a World Health Organization (WHO) scheme. That classification - a statistical average of test value, really - is what determines the treatment protocol. Our daughter's varitations from the statistical norm does not figure into the decision choices, we were advised, because any such variations would not be supported by existing laboratory and clinical trials. She was simply WHO Classification IV - SLE with glomerulonephritis - and that called for a particular treatment protocol, period. Researching this issue led us to Pediatric Kidney Disease, edited by Chester & Edelman, a comprehensive text (two inches thick, and weighs a ton) that discusses the WHO classifications in detail, as well as the treatment of glomerular disease using cytoxan and other immunosuppressive agents.
NETWORKING, NETWORKING, AND MORE NETWORKING.
Our broad scale networking efforts also began to pay off. We accelerated our learning process through numerous referrals to outside physicians - pediatric nephrologists and rheumatologists, immunologists in research and clinical practice, orthopedists, urologists, ob/gyn specialists, opthalmologists, neurologists, psychologists and psychiatrists, psychneuroimmunologists (mind/body physicians specializing in the immune system), and specialists in traditional Chinese medicine (TCM) and its Japanese counterpart (kampo). In particular, we made contact with a very special, very compassionate immunologist at the National Jewish Center for Immunology & Respiratory Medicine in Denver who teleconferenced with my wife and me almost every night, helping us to understand Hillary's prognosis and what Hillary's doctors were telling us; referring us to additional consulting specialists for special issues; and diplomatically helping us to build a cooperative partnership with Hillary's physicians.
A week after admission to Children's Hospital, Hillary was administered megadoses of methylprednisolone intravenously. Five days after that, she began her IV cytoxan treatments and hemodialysis procedures. These were the darkest days that my wife and I had ever experienced in our lives - watching our daughter's condition deteriorate by the day, looking to her physicians for hope, anxiously waiting for her lab results twice a day, every day, hoping...hoping...hoping.
Yet, in the face of this seemingly insurmountable challenge, Hillary's resolve shone forth like a beacon. She would not accept the doctor's prognosis, would not believe that she couldn't be
"outa there" in a week or two. She could not and would not accept the possibility that lupus would get her down, would prevent her from competing in gymnastics ever again, as her nephrologist had predicted. She was not holding out for mere remission, she told us in no uncertain terms; she wanted a cure, pure and simple. It's denial, more than one doctor pronounced, but they were all wrong. It wasn't denial at all... it was defiance.
EDITOR'S NOTE
The author prefers to remain anonymous for the present, and Hillary
is a pseudonym.
Hillary successfully weathered the crisis, and five months later, by
September 1993,
she began to show definitive signs of improvement. She resumed
classes at school, even though her body was frail, ravaged by the
powerful medications pumped into her. She doggedly returned to her
gym and began the slow, often painful process of rebuilding her body.
In February 1994, she attended her first gymnastics meet since her
lupus diagnosis, still too frail to tackle the Vault or Uneven Bars
or even Floor Exercises, but she walked away with 1st prize in
Balance Beam. The above article is adapted from an early chapter of
the book that the author is writing about his family's experiences
with Lupus.
From Lupus Letter Volume 1, No. 1
E. L. E. F.
European Lupus Erythematosus Federation