The Chronicles of Anemia—and a Frustrating Medical System - Pacific Standard
When my body stopped making enough blood cells, I entered the disconcerting world of doctors and hospitals, and found medical decision-making can be collaborative and empowering—except when it isn’t.

Until two years ago, I always considered good health to be my birthright.

I know that sounds arrogant, but I had evidence backing up my belief. My mother died at age 88, and my father is still going strong at 91. Despite growing up with the deprivations of the Depression, both managed to get through more than eight decades of life without a serious ailment.

Blessed with their genes, I considered myself, if not invincible, an unlikely candidate for illness. I avoided registering with a primary-care physician for years. But with a milestone birthday a mere year away, I decided it was a good time to get a check-up.

So began my — what to call it? “Adventure” is too lighthearted a word, “tragedy” too strong. Let’s go with the admittedly overused “journey,” since entering the medical system is like taking a short drive to a different world — one with its own set of rule and expectations.

Actually, “driving” isn’t quite the right metaphor, since, as a patient, you are never in charge of the steering wheel. I found there are points where you have some autonomy, and others where you have none at all.

Similarly, I witnessed incidents reflecting both awesome efficiency and maddening miscommunication. Medicine is a world of jarring juxtapositions — a mix of the miraculous and the mundane.

A bit of background. A few days after I turned 59, I saw a family physician, a kindly older man who has since retired. He reported that I was in good shape, with one itty bitty exception: All of my blood counts were abnormally low. A low red-cell count generally points to one type of disorder, while a low white-cell or platelet count suggests something else entirely. But across-the-board bad numbers indicated a serious systemic failure.

Within days, I had an appointment with a hematologist. The name on the door stopped me cold: “Ventura County Hematology-Oncology Specialists.” Oncology? Hold on here. Who said anything about cancer? Does this mean blood-related disorders are often cancers? (Spoiler alert: Yes.)

Thus began my ongoing relationship with Dr. Lynn Kong, whom I immediately found refreshingly non-dogmatic. She informed me that her first thought was aplastic anemia — a rare autoimmune disease in which the T-cells that spearhead and organize the body’s immune system go haywire, attacking the bone marrow that produces blood cells.

As with so many autoimmune disorders, there is no definitive test for aplastic anemia; it’s a rule-out disease. After a chest X-ray, abdomen ultrasound, two bone-marrow biopsies, and a wide variety of blood tests all came out negative, it was clear her initial diagnosis was correct.

This put me in a rather elite group, as aplastic anemia hits a mere two people in a million. (I couldn’t have won the lottery?) No one has any clue as to what causes it. Some genetic switch apparently just malfunctioned and sent my defense system on an errant, self-destructive mission.

The first-line treatment for this, I learned, was a week in the hospital, where you are injected for many hours each day with antithymocyte globulin. It’s a serum derived from horses which, as researchers discovered in the 1970s, binds with human T-cells and renders them inoperative.

You have turned over all your decision-making, even on the smallest level, to a set of professionals with an established protocol. That’s the deal: You do what they say, they save your life.

It is, in the understated words of one of my doctors, a “difficult drug” that can produce a range of side effects, from fever to chest pain to diarrhea. That, along with the thought of leaving my long-suffering spouse (who has her own chronic health problems, with multiple sclerosis), led me to seek out other options.

So under the guidance of an expert at the University of California–Los Angeles, I spent six months on a kinder, gentler drug, cyclosporine, on the one-in-three chance it would raise my blood counts on its own. No such luck. I was then placed on the list for the university medical center’s 36-bed blood-disorders unit (ATG is only available to such specialized facilities). The call came at 4 p.m. on a Monday to come on down or lose my space. I was checked in by 9. (Thankfully, I had good, employer-provided medical insurance that covered most of the cost.)

Among the cliches I can now confirm based on personal experience is “The first night in the hospital is the worst.” I had absentmindedly assumed I’d be more or less left alone until morning. What I failed to realize is that UCLA, like most hospitals, has its nurses on two shifts — 7 p.m. to 7 a.m. — and the night shift has just as many responsibilities to fulfill as its daytime counterpart. Whether you desire sleep is, basically, irrelevant. So I had blood drawn at 2 a.m., was awoken again to sign a consent form at 3, started getting a transfusion at 4 … you get the idea.

Around 6:30 a.m., the physician in charge of my care came in to introduce himself. He apologized for being earlier than usual — he had an early morning meeting to attend — but I was too groggy to coherently respond. I figured I’d have a better chance to talk with him on subsequent mornings — a notion which proved to be naive.

Each morning, he would stride into my room, listen to my heart and lungs, ask “What can we do for you today?”, and then immediately walk away. I came to think of him as “Dr. Sorkin,” since, like the similarly brainy characters of television dramatist Aaron Sorkin (The West Wing), his tendency was to walk and talk simultaneously.

If I was quick, I could get in one succinct question before he got to the door, which he answered directly before heading off to the next room. (When I mentioned this to one of the nurses, she noted that when he stays to talk with a patient, it usually means something is going wrong with their treatment. After that, I was happy to see him race away.)

That morning also saw the insertion of a line into a vein in my right arm, for easy delivery of both blood and drugs, and the binding of me and a metal stand from which hung various bags of intravenous fluids. Like my companion on a chain gang, this cumbersome contraption remained at my side for the next six days — bed, bathroom, even while taking a bath (an awkward situation, to put it mildly). It was all tolerable, because I was now set to start my treatment — as soon as the drug arrived. Which it didn’t, due to some confusion on the part of the pharmacy. I was given the rest of the day off.

But I was hardly idle. One thing I could not get used to was the parade of people in and out of my room — doctors, nurses, fellows, volunteers, cleaning staff, administrative personnel, and on and on. There is a clear protocol that hospital employees knock before entering, but there was normally no pause between the knock and the entrance. You hear a sound, look up, and somebody else is there, often with an apologetic smile and an explanation that they need to clean the door knobs, check your vitals, see if you would like some spiritual guidance, or (best case scenario) bring you a meal. (Food: not terrible, but bland.)

Concepts like “privacy” and “autonomy” are inoperative in such a facility, and “No, thanks” are meaningless words. You have turned over all your decision making, even on the smallest level, to a set of professionals with an established protocol. That’s the deal: You do what they say, they save your life.

Finally, a day behind schedule, the ATG treatment began. The stuff was pumped into me for four or five hours (it increased to up to 11 hours on subsequent days). I had no physical reaction at all, and I was feeling pretty good about myself for tolerating the “difficult drug” so well. Then, that night, my teeth started chattering. I had what the nurses call the “shake and bake” — chills followed by fever, which they treated with the surprisingly low-tech method of Tylenol and ice packs. This pattern continued for the rest of the week, with the shakes recurring during the third session (when they turned up the flow), and again after the fifth and final one.

My first week home was tougher than expected. I was told to get rest, and was prescribed 80 mg per day of a powerful steroid, which revved me up to the point where my nodding-off time was around 2 a.m. For the next three weeks, I was a daily visitor at my doctor’s office or a local hospital for a transfusion. And then, a couple of weeks later, I found myself back in the hospital.

I had been warned about the possibility of a bacterial infection, since the treatment involved suppressing a key part of my immune system. But I was under the naive impression that if it happened, I would get a prescription for some antibiotic, go home, and sleep it off. Instead, within a couple of hours from when I began shivering, I was being wheeled into the emergency room.

My first week home was tougher than expected.

Once there, I got a taste of what is, arguably, American medicine at its best. A swarm of medical professionals descended upon my curtained cubicle, taking blood samples, monitoring my vital signs, and starting me on an antibiotic drip. (At that point, they could only guess at what antibiotic would be most effective, but as it turned out, they guessed correctly.) Three or four hours later, I was assigned to a private room, where I would stay for the next four days and nights.

Mind you, my symptoms were gone by morning. But, as they told me each morning (to my increasing frustration), I wouldn’t be discharged until they figured out exactly what bacteria had gotten into my bloodstream, and decided how to treat it. (It turned out there were two: one common gut bacteria, and, inexplicably, one associated with cystic fibrosis.) I was relieved to be released, but being home meant spending another week attached to a machine — this time, a portable pump that injected me with an antibiotic once every six hours.

A year has now passed since my two hospitalizations, and while my blood cell levels are frighteningly volatile and, in some cases, far below normal, my energy is increasing. I’ve begun regular acupuncture treatments in an attempt to stimulate more production. My focus now is discovering how low I can go on my cyclosporin dosage without my cell counts collapsing. The calibration process is tricky, but I’m guardedly encouraged.

Throughout this ordeal, there have been times I felt empowered and listened to, and other times when the opposite was true. Dr. Kong, thankfully, was not defensive or annoyed when I brought in research I had found on the Internet (“At least you’re using good sources,” she said, conjuring up an image of patients presenting her supermarket-tabloid stories about miracle cures.) She explained my options clearly and helped me decide how to proceed. My treatment felt like a genuine collaboration.

The hospital physicians were a different story. As a rule, they seemed uninterested in dialogue, or any information a test didn’t provide. If my experience is typical, the concept of empowering patients, and allowing them to take an active role in their treatment — which is big in the medical literature — has yet to fully catch on. At least in some settings, that “we’re the experts” attitude is as resilient as antibiotic-resistant bacteria.

This is more of an observation than a complaint. But there is something bizarre about having a vast staff surrounding you, but finding that no one except the nurses (whom I deeply appreciate and admire) has the time or inclination to tell you what’s going on.

I have no doubt hospital doctors work hard and are ridiculously busy, but opportunities for dialogue must somehow be built into the system. Tests, after all, don’t always tell the whole story; a more holistic approach would surely give a more rounded picture of both patient and disease.

And while good health may not be anyone’s birthright, being listened to, and given full explanations regarding our treatment, is surely our due.

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