Reflections on Living with Lupus, and on Treating It

There are some people whose purpose in life is to entertain. Some of us are fortunate to have them as our patients. One of my favorites is Larry the Cable Guy. Before you get all excited and report me for a HIPAA violation, I am not referring to that redneck comedian with his own weekly television sitcom and a host of B-rated movies. My guy Larry was training to becoming a real cable-installation guy when one day he felt awfully short of breath. Really, really breathless. As though someone was wringing his chest like a wet sponge. He thought he was going to die. He nearly did. He was rushed to a nearby hospital and brought straight to the operating room where he underwent a lifesaving pericardiocentesis procedure. This brought Larry some temporary relief, but two days later the fluid had reaccumulated and his blood pressure began to drop. After a few more procedures to strip his heart of its pericardial lining, his condition stabilized. Larry was nineteen and thought he was following the path of his older brother who had died a year earlier from complications related to myasthenia gravis.

At first, his doctors believed that he had been exposed to a virus. Isn’t that what we tell patients when we really don’t know what their diagnosis is? His chest X-ray showed a large, globular, swollen heart bordered by a pair of healthy-looking lungs. There were no infiltrates, no pleural effusions. The blood test results demonstrated the expected moderate rise in his white-cell count, but none of his many blood and body fluid cultures grew any microorganisms, so infection seemed unlikely. His kidney and liver function studies were normal, so that ruled out organ failure as the cause of his pericardial effusion. Then came the surprise. A careful microscopic search of a sample of pericardial fluid provided the missing clue: the mysterious lupus erythematosus cell, better known as the LE cell.

The LE cell-prep test is based on the innate ability of some lupus monocytes to phagocytize cell nuclei rendered antigenic by the disease. To this day, the finding of an LE cell remains the most specific yet the least sensitive test for diagnosing lupus. Nobody ever gets to see the LE cell anymore, except when it makes a cameo appearance during some esoteric discussion of a clinical–pathologic case. There is a good reason for its demise. First, you must shake and stir your sample fluid with some glass beads in order to disrupt cells to get them to release their nuclear material. Then you have to carefully gaze at dozens of microscopic fields to try to find the elusive white blood cell with the bulging cytoplasm that looks as though it ate far too much at the all-you-can-eat buffet. And so began Larry’s lupus journey.

Larry’s episode of cardiac tamponade never recurred. In fact, after tapering off the three-month course of prednisone that was prescribed to him, Larry decided that he was ready to move on. He packed his bags and moved to Hawaii, where he worked as a cable guy and life was good—work six days then relax on the beach and take in a little sun. As I learned, Larry defied many of the rules that we spell out to our lupus patients. He just thought that he looked better with a suntan. I guess who doesn’t? But the fun times did not last forever. He began to notice some difficulty walking. He tired very easily. Eventually he had to give up his job because of his troubles. It was time to come home to Boston.

Larry’s Lupus Rears Its Head

This is when I first met Larry. I quickly learned that he was a man with many friends. If I ever needed help with parking ticket citations, Larry assured me that he had people in the “right places who could help make them disappear.” Was I looking for Sox or Bruins tickets? No problem. He had friends working in all the Boston teams’ box offices.

Larry did not look well. He limped around my 60-square-foot clinic office, clutching the furniture for support. His hip X-rays revealed those telltale signs of avascular necrosis (AVN); a tiny sclerotic rim capped each femoral head. My orthopedic colleague Tom Thornhill suggested we consider a new treatment for AVN proposed by Drs. Tom Zizic and David Hungerford of Johns Hopkins University in Baltimore. They and others postulated that increased venous pressures may be contributing to the development of AVN. Their technique involved reaming out a core of femoral bone marrow and cortical bone to reduce the vascular pressure in the femoral head. Years later, I was amazed at how this procedure helped reduce Larry’s pain and allowed him to preserve reasonable function even when his subsequent X-rays demonstrated a loss of cartilage space in both hips.

However, there were other serious problems to contend with. As Larry’s story taught me, with lupus there is no limit to how many things can fall apart at once. Out of the blue, his blood-cell counts dropped—whites, reds, and platelets. His kidneys, which had functioned well until now, were starting to fail. Larry was a short guy, but the excess edema and weight gain from the steroids that we prescribed made him look even squatter. However, Larry still kept his biting sense of humor. I usually could tell when he had arrived at the clinic. He was always at least 30 minutes early, which I found unnerving, because I tended to run at least 30 minutes late … on a good day! The waiting room was Larry’s stage. Within minutes, he would have all the other patients in stitches. His impersonations of the staff were uncanny. Patients didn’t seem to mind if their doctors were running late. In fact, some of them requested that others go ahead so that they could stay to watch Larry’s whole show.

Unfortunately, the news about his lupus was no laughing matter. The renal biopsy demonstrated a diffuse proliferative glomerulonephritis. Coupled with his hematologic issues, we decided that the best treatment option would be cyclophosphamide and an escalation in his steroid dose. Larry carefully read the brochure about cyclophosphamide.

A confirmed bachelor, he assured me that he was not worried about fertility issues. When he came across the term “anorexia or loss of appetite” he chuckled. “That’s not a side effect, that’s a benefit, right?” he asked.

Treatment Success—For a Time

Fast-forward another seven years. He tolerated the cyclophosphamide, prednisone, azathioprine, and all the other drugs we used to treat his lupus. Along the way he lost 130 pounds, about one-third of his original body weight. Larry kept the belt that he wore when he weighed more than 370 pounds. As his weight began to drop, he would tighten the belt clasp until he moved it up by about 10 inches. His goal was to be able to wrap his belt around himself twice. I suggested that he consider selling a version of his “weight reducing” belt on some television shopping network. “Only if you sell it with me!” he replied. Perhaps we should have.

The good times did not last long. One Friday morning, Larry paged me to let me know that he was having a problem with his vision. He described it “as though I am missing one half of my visual field in either eye.” I was attending a daylong conference, so I told Larry to come to the emergency room (ER), and from there I expected that he would be admitted to the medical service. I came by the ER later that evening but couldn’t find Larry. He had been discharged an hour earlier because the ophthalmology resident felt that this problem was related to his lupus and could be treated as an outpatient. What the exact problem was though, he couldn’t say. Six hours later, I admitted Larry to our service. Over the next few days it became apparent that his homonymous hemianopsia was caused by a thrombotic event due to a sudden spike in his anticardiolpin antibody titer. This titer had been in the single-digit range six months earlier. Now it measured over 140 units.

Not surprisingly, Larry’s vision did not improve. One day he brought along some paperwork for me to fill out. Despite his connections, he was having “some problems” getting his driver’s license renewed. Not surprisingly, he failed the eye exam and wanted a letter from me stating, nonetheless, that I thought his vision was sufficient to drive a car. I told Larry that the registry would want such a letter to be written by an ophthalmologist and not a rheumatologist. We didn’t discuss this issue again for several months until I asked Larry how he was getting along without a car. I could sense by the look on his face that I was going to be surprised by his response. “Hey, I just added a couple of extra-wide mirrors on both front doors and I try to drive in the right lane. Doc, there are far worse drivers than me out there!” If you have ever driven in Boston, you’d probably agree.

A few years later, for the first time, Larry missed his appointment with me. I knew that something must be terribly wrong when I failed to see him holding court in the waiting area 30 minutes before his appointment. I tried calling his home phone, but the line was constantly busy. About an hour later, I received a call from his mother, who told me that she’d found Larry dead in his bed. An autopsy was not performed, so I can only guess that Larry died of a cardiovascular event.

Larry’s Lessons

What did I learn from Larry? Perhaps the most important lesson was to follow the sage advice of his favorite philosopher, the baseball legend Casey Stengel, who once decreed: “Never make predictions, especially about the future.” This was Larry’s credo; he always lived life to the fullest. Never mind that he faced a life-threatening cardiac tamponade, or that a few years later he would be told that lupus nephritis would eventually destroy his kidneys and lead him to the dialysis unit. And that, for twenty years, somehow he kept walking on a pair of totally worn out hips that should have been replaced a decade earlier. And how he only could see half of his visual fields. Nothing fazed Larry. Was it a strong sense of denial? Maybe. But I always knew that he understood the serious nature of his disease. He just wanted to imagine a better outcome. He once confided that he used positive imagery as a way to create a world where he could live his life as though he had no illness.

Larry was not a therapeutic nihilist. He would have only partially agreed with the patient–author of Anatomy of an Illness, Norman Cousens, who once said that, “drugs are not always necessary, [but] belief in recovery always is.” Larry recognized his need for treatment. Yes, be optimistic, but don’t leave everything to chance. When I look back at Larry’s treatment and compare it to what we would have offered him today, there is literally no difference: steroids, azathioprine, hydroxychloroquine, cyclophosphamide. Sadly, not much has changed over the past 40 years of lupus therapeutics. Yes, we are better at treating the infectious complications that sicken our lupus patients and maybe we are improving the management of their cardiovascular risks. But we owe it to Larry to do a far better job at taming the lupus beast.

Dr. Helfgott is physician editor of The Rheumatologist and associate professor of medicine in the division of rheumatology, immunology, and allergy at Harvard Medical School in Boston.