Lupus: An ongoing medical mystery / by sylvia kronstadt
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|Mon, 05-27-2013 - 8:37am|
Lupus: A Rash Quest for the Truth
In spite of all the dire warnings I’ve received, I am still refusing to take the medications that have been prescribed for my condition. My decision to do the best I can to take care of myself has been quite liberating, and I am at peace with it (most of the time).
I have become more convinced that probiotics are helping me, and I have found medical evidence that supports my inadvertent discovery. I will elaborate below.
I endured a florid, unsightly and uncomfortable rash under my eyes for nine months, starting in May 2010. Like so many of you who have responded to my blog post, I have no idea how sick I am or how sick I may become.
TANGLED IN UNCERTAINTY
I am constantly wondering what will happen next, and I have failed to find a credible source of information or reassurance. My most basic questions to several doctors were met with outrageous evasion.
( If they don’t know, I wish they would just say so. Although if a doctor doesn’t know the answers to questions as basic as mine, I think he ought to educate himself or find another, less taxing, line of work.)
A number of you have asked whether the probiotics I am using have continued to suppress my rash.
MY MIRACULOUS REMISSION ENDURES
I have stressed that I can’t be sure the probiotic had anything to do with the complete disappearance of my rash within 36 hours after taking the first caplet. But the remission was so quick and complete -- after so long -- that I am continuing to take it and to have some faith in it.
The more I read, the more I believe it might have precipitated a major change for the better in my condition.
12 LITTLE STRAINS RELIEVED MY BIG STRAIN
I had been taking an inexpensive probiotic for more than 20 years, which contained only two strains of lactobacillus. The product that seems to have had such a dramatic effect has 12 strains, including lactobacillus and several others.
‘YOU COULD BE ON TO SOMETHING’
When I told my primary care doctor that it was “probably just a coincidence” that my rash vanished so quickly after I took the new probiotic, she said, “Why do you think it was a coincidence? You could very well be on to something.”
From what I have since learned, I am outraged that not one doctor suggested I try probiotics. The theoretical basis for doing so is solid, and reputable medical institutions acknowledge it.
PROBIOTICS REGULATE THE IMMUNE SYSTEM IN A PROFOUND WAY
Probiotics have been used for thousands of years, according to an article in the February 2004 issue of the journal Gastroenterology.
Researchers at the University of California, San Diego (UCSD) School of Medicine and the Shaare Zedek Medical Center in Jerusalem, Israel report that people in ancient Babylon used the live organisms to maintain health. The body’s immune system is based in the intestinal tract.
GENTLY CALMING THE IMMUNE RESPONSE
I have recently found additional data that seem to support the use of probiotics to modulate the immune system. In discussing autoimmune disorders, the Mayo Clinic says “probiotics may help calm the immune system's overreaction in ways not fully understood.”
A study carried out at the University of Helsinki in Finland found that probiotics can exert “specific immunomodulatory effects” and have an anti-inflammatory potential (as reflected by reductions in CRP), which could be very pertinent to the lupus patient, whose body is flooded with inflammation.
KEEPING MILD AUTOIMMUNITY FROM EXPLODING
The Life Extension Foundation recommends probiotics for the lupus patient, along with Vitamins C, B12 and D3 and Omega-3 fatty acids (I take all of these and many more, as I have been for decades).
An osteopath whose opinion I value said just last week during his daily TV program that if tests indicate you are beginning to develop autoimmunity, probiotics can help suppress it, preventing it from becoming a full-blown autoimmune disorder.
THE FUTURE OF MEDICINE -- LET’S TRY IT NOW
The beneficial microbes in probiotics could represent “the future of medicine,” with the potential to treat a variety of diseases in humans and animals “from diarrhea and eczema to gum disease and autoimmune disorders,” according to a report released by the American Academy of Microbiology in 2006.
“Probiotics could also conceivably be put to use in preventing disease or thwarting autoimmune disorders. A number of these possibilities are being explored in research labs and hospitals around the world," says Richard Walker of the Food and Drug Administration, a co-chair of the steering committee that produced the report.
LIKE A SNEEZE, BUT A BILLION TIMES WORSE
We do know that autoimmunity is a hypersensitivity reaction, similar to an allergic response. In allergies, the immune system reacts to an external substance that it normally would ignore. With autoimmune disorders, the immune system reacts to normal body tissues. There are an estimated 80 to 100 medically recognized autoimmune disorders.
THE INTERPLAY OF GENES & ENVIRONMENT
Between 1.4 million and 2 million people have been diagnosed with lupus, about 325,000 in the U.S. The disease affects 1 in 185 Americans. Although the cause is unknown, scientists suspect that people are genetically predisposed to lupus.
They also believe that environmental factors, including infections, antibiotics, ultraviolet light, extreme stress and certain drugs play a critical role in triggering lupus.
I was surprised to learn that a certain level of autoimmunity is normal.
WE’RE SUCH COMPLAINERS, THEY CAN’T STAND US
According to a 2001 survey by the Autoimmune Diseases Association, over 45 percent of patients with autoimmune diseases have been labeled by doctors as “chronic complainers” in the earliest stages of their illness.
(That attitude is so typical of the medical establishment, which is one of many reasons I try to avoid doctors entirely.)
IGNORED, DISPARAGED, SENT PACKING
“This can be devastating to a young woman who may then begin to question her sanity as she tries desperately to find out what is wrong. Tragically, many of these patients suffer significant damage to their organs in the meantime and end up carrying this health burden with them for the rest of their lives because of the delay in diagnosis,” according to the American Autoimmune Diseases Association.
“There is very little focus on basic autoimmune research, even though one in five Americans has an autoimmune related disease,” it adds.
I MIGHT AS WELL HAVE TRIED VOODOO
After trying unsuccessfully to treat my swollen, shiny, bright red rash myself for four months, using a naturalistic “What Would Cleopatra Do?” approach, I spent the next five months seeing doctors and submitting to tests.
Despite all the time and money I invested in conventional medicine, I never got a definitive diagnosis, prognosis or treatment plan.
DOCTORS: THE GREAT PRETENDERS
Lupus has been called the “great imitator.” The medical establishment should be thought of as the “great pretender.”
I have had two doctors diagnose me with systemic lupus erythematosus (SLE); two have said that it is cutaneous lupus; one referred to it as a “nonspecific autoimmune disorder”; and another deemed it “multiple connective tissue disorders.” My lab results were characterized as conclusive for SLE by some doctors and as “suggestive” by others.
MY SKINNY SKIN SKIN
Even my biopsy provoked argument, with one dermatopathologist declaring that it indicated lupus; another -- who is a specialist in lupus -- said it revealed “inflammation in the upper skin with different types of immune cells but not in a pattern that was specific for any particular diagnosis.”
As I wrote in March of this year, the rheumatologist who so confidently and sweepingly diagnosed me with systemic lupus and “multiple connective disorders, including fibromyalgia,” Dr. Shruti Sanghvi, reversed her diagnosis two months later, saying only that I no longer met the criteria and that I did not have lupus, even though my blood work revealed the same degree of autoimmunity.
WAS SHE WRONG BEFORE OR IS SHE WRONG NOW?
I was completely baffled. As far as I could tell, nothing had changed. Was she saying that my body had cured itself of lupus, or that I never had it? She had initially urged me to begin taking drugs immediately.
My kidneys could fail at any time, without warning, she said, and the autoimmune process could also severely and irreversibly damage other organs, including my brain. Even after withdrawing her diagnosis of SLE, she told me I should take medication to protect myself.
According to a May 4, 2012 article in Medical News Today, "Evidence indicates that 35% of adult Americans have evidence of lupus nephritis (diseased kidneys, leading to renal failure) at the time of SLE diagnosis, and 60% of patients with SLE develop nephritis during the first decade after SLE diagnosis." About 12 percent die within the first 10 years after diagnosis.
DO THESE PEOPLE HAVE NO SHAME?
Protect myself from what? I was left speechless and dumbstruck as she blithely withdrew her initial diagnosis and swept out the door. When I had time to gather my wits, I sent her some questions about what this all meant, and she ignored me. They will be included in my next post, which discusses autoimmunity in general.
I was urged to use one medication by two doctors and aggressively warned against using it by two doctors, a pharmacist and the medication’s own web site.
IF ONLY I ACTUALLY DID HAVE MALARIA!
Several doctors urged me to take an anti-malarial drug for the rest of my life, despite the lack of a diagnosis, because it “might provide some protection against flares,” if indeed I actually have a condition that produces flares.
Yet another doctor, Brian Williams, said the anti-malarial drug is “much worse” than prednisone -- that it would turn my skin blue-gray and destroy my retinas. (I have since learned that this is a gross exaggeration, to put it mildly.)
(Late in 2011, I felt the need to begin taking Plaquenil, for several reasons.
WE ARE UNIFIED BY OUR PROFOUND BEWILDERMENT
I have received feedback from all over the country and from several foreign nations, many of them desperately posing the same questions that I am still asking.
LUPUS LURKS, BUT I’M STARING IT DOWN
My skin has looked fine since February -- and there was no scarring -- but the lupus (or whatever it is) still lurks below the surface like a pool of blood, and when I gently apply Vitamin E to the area (which is the only moisturizer I use now, except for Vaseline at night), the redness floods right to the surface. It goes away quickly and is rarely uncomfortable.
My persistent rash never had the malar formation that is characteristic of SLE. It was under my eyes and did not extend across the bridge of my nose, unlike the malar profile.
MALAR REARS ITS BUTTERFLY HEAD
Recently, however, whenever I get up from a nap, my nose and upper cheeks are very red -- much closer to a malar formation than I’ve ever had. Even though it fades within a couple of hours, it concerns me.
Although repeated retesting has indicated ongoing elevated levels of anti-nuclear antibodies (ANAs), double-strand DNA and anti-chromatin antibodies -- which are characterized as “frequent early markers of systemic lupus” -- I have yet to receive a credible diagnosis of any sort.
GOING IT ALONE -- NO BIG PHARMA FOR ME
So far, I have taken no drug for my condition during its 14-month duration. I have continued to adhere to my extremely healthy, vegan, anti-inflammatory diet (see Dr. Andrew Weil's web site for this diet's "pyramid.") I jog every morning and do weight training several afternoons a week. I take lots of nutritional supplements, as I mentioned earlier. I stretch and do yoga.
I was doing all of this for many years before I acquired an autoimmune condition, so perhaps it seems silly to think that it will do me any good at this point. But I haven’t relinquished the hope that my body can take care of itself.
I have felt uncomfortable with the idea of taking a powerful pharmaceutical agent without even knowing what is wrong with me.
MAYBE I’M BEING STUPID… I GUESS WE’LL SEE
Sometimes, I become uneasy with the path I’ve chosen. The internal “flares” that one can have and be completely unaware of them do concern me. These flares can severely damage the body, especially the kidneys, the brain and spinal cord and the heart and lungs.
Information from reputable medical sites says that even the most minor exposure to ultraviolet radiation is one of the “triggers” for such flares. The anti-malarial drug that I have been urged to take can be “protective” to some degree, they say.
TO 'D' OR NOT TO 'D', THAT IS THE QUESTION
I was unable to understand some of the pertinent data I came across.
We have all heard reports of study after study indicating that Vitamin D supplementation is important in many ways, including the suppression of autoimmune diseases and cancer. We have repeatedly been warned that there is an 'epidemic' of Vitamin D deficiency in the U.S., even as more is learned about its remarkable benefits.
A study last year by researchers at Harvard and Oxford, led by Sreeram Ramagopalan, buttressed this position, recommending supplementation as a preventative strategy in these diseases, and noting that that vitamin D receptor binding is significantly enhanced in regions of the human genome associated with several common autoimmune diseases.
However, a 2009 study postulated that “supplementing with vitamin D may actually exacerbate autoimmune disease.” The paper goes on to point out that “molecular biologists have long known that the form of vitamin D derived from food and supplements, 25-hydroxyvitamin D (25-D), is a secosteroid rather than a vitamin. Like corticosteroid medications, vitamin D may provide short-term relief by lowering inflammation but may exacerbate disease symptoms over the long-term.” The leading spokesman and theorist behind this research, Dr. Trevor Marshall, explains that by deactivating the VDR and subsequently the immune response, 25-D allows bacteria to spread more easily in the long-run. He believes that the conventional wisdom about the value of Vitamin D supplementation is a growing public health hazard. I am not qualified to convey his ideas coherently, but you can read about them at trevormarshall.com/BioEssays-Feb08-Marshall-Preprint.pdf
I LOVE VITAMIN D!
I have more than doubled my intake of Vitamin D in the past couple of years, and I’m not sure how to respond to this study. I have been unable to find research that explains, supports or contradicts the Marshall team's conclusions. They note that both Vitamin D supplementation and autoimmune diseases have risen sharply in recent years, but they do not provide any evidence of causality. Even so, his larger theory about the role of pathogens in the Human Microbiome is fascinating.
At this point, I've decided to compromise and cut my Vitamin D intake in half.
OPENING A CAN OF HELPFUL WORMS?
In research being conducted at Stanford, and around the world, PARASITIC WORMS have been found to reverse autoimmunity and return the immune system to its normal functioning. Remarkable results have been seen in Multiple Sclerosis and Crohn's disease, and it is likely that it will be effective for other autoimmune disorders, including lupus.
ONWARD LUPUS SOLDIERS
I am going to continue my battle to be as healthy as I can be, and I will persist in learning as much as I can about autoimmune disorders. It’s actually a very interesting subject.
In the meantime, I am just grateful that I don’t have one of the many chronic and/or progressive conditions that are much worse than whatever the hell it is I have.
I am also grateful to know that there is such a dynamic, well-informed, generous and compassionate lupus community out there. What all of you are doing to help yourselves and each other is truly awe-inspiring.
Does lupus make everyone ugly, or is it just me? http://kronstantinople.blogspot.com/2012/10/lupus-blushing-terrorist-bulges-with.html
A Lupus Mystery
My battle with lupus, or whatever it is
(March 20, 2011) The tiny, burning red dots began appearing under my eyes as I held Daddy’s hand in St. Mark’s Hospital during his final days. After he died, on May 10, 2010, and everyone else had left, I bent over to hold him, and the sobbing began. I stayed there, convulsed with grief, until the most beautiful, soulful nurse I have ever met, Veronica Mead, embraced me tearfully and told me I had to go. I removed his dementia bracelet, and I’ve worn it ever since. On the back, it has his name and phone number. On the front, it says, “Safe Return.”
I have never cried so hard and long in my life, and I still have a lot of it left to do. Within a week, the red dots had formed a bright, raised crimson half circle under each eye, with stray dots down my cheeks and into my temple. It was so swollen that I could see it just by looking down.
For me, going to a doctor is always a last resort -- it's generally a total waste of time and money. For three months, I took a “What Would Cleopatra Do?” approach to my rash, which had become more inflamed and monolithic. I tried yogurt, honey, olive oil, aloe, Vitamin E, flaxseed oil, cucumber slices. I tried nutritional supplements such as beta glucans, oil of oregano and olive-leaf extract. When natural remedies didn’t help, I tried antiviral, antifungal and antibacterial ointments. I dissolved aspirin in water and tried in vain to get an anti-inflammatory effect from it.
By then, it was starting to scare me. I was afraid that if it ever went away, it would leave significant scarring. So I arranged to see Salt Lake City dermatologist Brian Williams.
Hippocrates on his head: First, do no good
Dr. Williams strode into the examination room 55 minutes late, twirled toward me, and almost immediately declared, with a thrilling trill of triumph: “I know what you have! You have lupus!”
He ordered lab tests that he was confident would prove the diagnosis he had boldly pounced upon – lupus -- and said a biopsy would probably be required later.
I was devastated at the prospect of living with lupus. It is an autoimmune disease that can manifest itself merely as a florid rash (cutaneous lupus) or it can also attack the body’s internal organs, including the liver, kidneys, heart, bones and brain (systemic lupus). There has not been a new drug approved to treat this disease in 50 years.
“Is there anything I can do to prevent scarring in the meantime?” I asked. It’s perversely coincidental that the feature people compliment me about the most -- the smooth skin under my eyes – is the very part of me that was being eaten alive by this flaming monster.
“Oh, there’s scarring already -- I can see it,” the doctor replied cavalierly.
Three weeks later, Dr. Williams called to inform me that the lab work supported his diagnosis of systemic lupus, although the data weren‘t 100 percent conclusive. The report indicated elevated Anti-Nuclear Antibodies (ANAs) and Double-Strand DNA levels, as well as liver and kidney abnormalities -- all key markers for the disease. The ANAs indicate auto-immunity: Your immune system, instead of attacking alien invaders as the Good Lord intended, attacks you.
Ironically, I love my immune system, and have for years done everything I could to build it into a Big Mean War Machine, poised to decimate any bug that was reckless and tasteless enough to enter the domain of my body. Now I felt like a benevolent dictator whose army has inexplicably turned against me. There used to be a band in Britain called “The Kronstadt Uprising,” and that phrase resonated with the chaos I imagined unfolding in my bloodstream.
Two therapists I know, one here and one in New York, said they suspected that this outbreak was triggered by my father’s death. Not by all that crying, which had been my first thought, but by the grief. They said that emotional trauma and other stressors can trigger an autoimmune disease in someone who is predisposed.
My blood pressure and heart rate, which had been very low all my life, were dramatically elevated, and my blood oxygen level, which had always been very high, was disturbingly low. Dr. Williams urged me to see Christopher Hansen -- a University of Utah dermatologist who specializes in cutaneous lupus – as well as a rheumatologist, who would address the systemic aspects of the disease.
“I am not going to take prednisone,” I declared. This was the most commonly used drug for lupus, I had read, and it didn’t seem worth it to me. It essentially kills your immune system – leaving you vulnerable to every bug out there, and to cancer as well – and it has an array of other side-effects, including bone loss.
“No, you’ll be on anti-malarials, which are much worse,” Dr. Williams said with apparent enjoyment. “They turn your skin blue-gray and destroy your retinas.”
(Note to the American Society for Psychiatric Dermatology – if there isn’t one, let’s get going on it – Please do an assessment of this doctor’s sadistic tendencies. He seems to relish delivering bad news, and he makes no effort to comfort or reassure the patient. Does he do this with everyone, or did I just bring out some evil streak in him, remind him of his emasculating mother, a luscious-but-treacherous ex-wife, etc.? For some reason, I am actually fond of the guy, but I do think some intervention is called for.)
You Want a Piece of Me? Just a tiny one
Dr. Christopher Hansen was fully booked for two months. I was unwilling to wait that long to get a biopsy and some more input on this whole lupus specter.
I made an appointment to see Dr. Doug Woseth. He is a very kind, patient, gentle, modest and somewhat shy young man. No bit fat doctor's ego for him -- he is just plain sweet. He laughs with a slight discomfort, which only adds to his considerable charm. He is so clean that one wants to hug and smell him. He delicately examined me and said he was quite certain that I didn’t have systemic lupus, but he cut out a sliver of my inflamed skin to determine whether I had the cutaneous form.
Dr. Woseth persuaded me to try the triamcinolone steroid cream that I had refused when Dr. Williams suggested it. Steroids are notorious for causing skin thinning, and the skin around your eyes is already the thinnest on your body. Even the manufacturer warns that it should not be used on the face “and particularly not under or near the eyes.” It can literally cause the skin to tear and shred. I was aware that it is widely prescribed anyway, to treat persistent lupus rashes, but I was concerned.
Dr. Woseth said, “It won’t hurt to use it for a couple of weeks to clear this up.” So I got some. When I took off my sunglasses and showed the pharmacist where I was going to apply it, she said, “You have got to be kidding. Don’t do it.”
Steroids are as effective as they are destructive. By this time, my rash had been in full flare for more than four months, but there was substantial improvement within days of beginning the triamcinolone. I have since learned from my research that many people with lupus and other serious skin conditions are initially delighted with the healing effect of the steroid cream, but then as soon as they stop using it – and you do need to use it sparingly and briefly – their condition roars back, full-force.
My biopsy results came back a week later and were “consistent with Lupus erythematosus,” according to the pathologist.
Doctor Dreamy Dares to Doubt
The Golden Boy of cutaneous lupus, Dr.Christopher Hansen at the U., got a break in his schedule, and I saw him a few days later. He told me that I needed to stop using the steroid cream immediately. The damage that I had feared apparently was occurring already; he mentioned skin atrophy and telangiectasiae (dilated superficial blood vessels). A visible, burning slit was developing at the outer corner of each eye as the tissue degraded.
The youthful Dr. Hansen is so thoughtfully “designed” as a physician that he almost seems like some sort of avatar. He is everybody’s dream doctor: handsome, earnest, authoritative, reassuring. Someone has programmed him with “excellent eye contact” software, which is quite lifelike. He managed to be extremely businesslike, enforcing the proper professional distance, while also conveying what seemed to be honest compassion. He asked lots of questions (very good ones), listened well, scrutinized and examined me (after washing his hands, thank god -- how often does that happen?) He even inquired about my professional and personal history and my current life, as if he actually cared who I am as a person -- almost unheard of these days. It was clear that he was really thinking – trying to solve this puzzle and to help me -- which ought to be the norm in the medical profession but most certainly is not. His pat on my shoulder was not the norm, either, and I appreciated the gesture. When I asked him a couple of questions about himself, he responded in the royal “we,” which could have reflected either grandiosity or humility, but either one was fine with me.
He ordered more lab work and said he wanted to examine the lupus-positive biopsy himself. He had his doubts that my rash was cutaneous lupus, and he was even more doubtful that I had systemic lupus.
Within 36 hours of discontinuing the steroid cream, as per the doctor’s instructions, the rash had flared back alarmingly. Even so, I was glad I wasn’t using the product anymore. I asked Dr. Hansen to prescribe a nonsteroidal anti-inflammatory ointment for me to try, even though his assistant had told me that the so-called TCIs aren’t very effective.
I began applying the Protopic ($130 per tube) but the inflammation it caused was intolerable, so I had to stop. The rash was creeping back in a whole new configuration, evolving from a broad red swath under each eye into an ever-changing assemblage of dense, small, very uncomfortable red bumps. They burned and itched, and it felt as if tiny worms were emerging from my pores. Each day, when I couldn’t stand it any longer, I knocked myself out with tranquilizers and got into bed with my cat.
Dr. Hansen emailed me about the lab work and said, “I see no evidence for any systemic involvement of lupus…I believe the risk of you developing systemic lupus based on your labs to be remote.”
He still had not received my biopsy, which he reiterated that he wanted to assess for himself.
At this point, having endured this painful and grotesque malady for more than five months, I still had no definitive answer. One doctor believed I had systemic and cutaneous lupus; one believed I had only the cutaneous form; one wasn’t at all sure I had either one.
A Queen of Karma, Bereft of Benevolence
While I waited for Dr. Hansen to re-evaluate the biopsy, I saw rheumatologist Dr. Shruti Sanghvi at the Salt Lake Clinic. I chose her for reasons my late father would probably describe as reverse discrimination. I chose her because she is from India, and in my experience, people – especially doctors – from India are particularly intelligent, competent, empathetic and charming.
I later thanked her for reminding me that GENERALIZATIONS ARE STUPID (although I continue to cling uncritically to my open-hearted love for black and hispanic people).
She floated into the examination room as if she were the Goddess of Healing, with her white lab coat over a violet sari. She had a subtle hint of mirth and serenity in her large eyes, and a comforting fullness to her face and body that reminded me of Buddha, which I know is the wrong country and the wrong religion, but that’s what I saw. She expressed a lighthearted curiosity about what made me tick. She said she admired my vegan diet and my exercise regimen, and she found the fact that I had embraced this lifestyle while I was still drinking heavily and smoking to be quite amusing.
After briskly skimming through my blood work and biopsy results, and then pressing a few joints on my fingers and toes, and sweeping her arms around me, as if she were reading my aura, she declared that I had “multiple connective tissue abnormalities” and diagnosed me with both fibromyalgia and systemic lupus. She urged me to begin taking an antimalarial drug right away. “You could have kidney failure at any time, without warning,” she said. “You could wind up on dialysis. You could wind up on a transplant waiting list. Even if the rash goes away, the disease can flare internally and damage major organs catastrophically.”
I should have been devastated, but in my gut, I didn’t believe I had lupus. Her New-Agey examination had seemed like something out of “Saturday Night Live.” I asked the doctor what made her so sure. She showed me a list of criteria that are used to diagnose the disease and said that if a patient meets four of the criteria, it indicates lupus. When she pointed out which criteria applied to me, I was even less convinced. Each symptom was so minor, and could have indicated so many other conditions, that it simply wasn’t persuasive. The only exception was what is known as a “malar” or “butterfly” rash, which is so distinctive that it is a very strong indicator of lupus. I told her that I never had this formation, which extends over the bridge of the nose and down onto each cheek. I showed her a picture of my rash at its worst, and it clearly wasn’t malar. She seemed taken aback but didn’t retreat from her diagnosis. She gave me no resources to deal with this very complex and amorphous disease, except for a pamphlet from the Lupus Foundation.
As she left the room, she said, “I am very sorry this is happening to you. It must be due to Karma.”
I rushed to open the door and called out down the hall, “Doesn’t Karma mean I did something to deserve it?”
She turned, smiling inscrutably, and replied, “Maybe in a previous life.”
A few days later, I informed her by email that Dr. Chris Hansen had just emailed me his own assessment of the biopsy: “There were some features suggestive of lupus but I would not make the diagnosis based on the biopsy alone, There is inflammation in the upper skin with different types of immune cells, but not in a pattern that is specific for any particular diagnosis. The diagnosis remains a mystery.”
Nevertheless, Dr. Sanghvi never expressed any second thoughts about her diagnosis. Since I refused to take the antimalarial drug, she suggested that I come back in two months for more lab work, to assess my level of autoimmunity and to monitor my kidney function.
Brash Questions, Rash Indifference
By November 2, I had lived with the rash for six months. Upon reviewing my new labs, Dr. Sanghvi announced that I did not have lupus after all and left the room. I was stunned by this sudden, inexplicable turnaround. Her positive diagnosis had never seemed credible to me, but then neither did this negative one. I felt totally lost and confused. I mailed her some questions, hoping to get a clearer picture of where things stood:
My Anti-Nuclear Antibody numbers, as we discussed, continue to be abnormally high. What are the implications of this?
Does the elevated ANA mean that I have an autoimmune disease of some sort, even if it’s not lupus? If not, what does it mean?
Do I need to be retested regularly?
Does my kidney function need to be monitored?
Is the rash under my eyes likely caused by the ANA issue, or do you think they are separate phenomena?
Since I exhibited enough of the criteria in September to be diagnosed with lupus but not enough in November, is it possible that in January I will once again face the prospect of a lupus diagnosis?
There are several other readings that are out of range and that I don't understand, but the most concerning for me are the references to my “high chromatin antibody level,” which is described in the report as a frequent "early marker" of systemic lupus. Shouldn’t I follow up on this?
She never replied. I had paid her well over $400, and all I'd gotten was two contradictory diagnoses, each delivered with an air of absolute confidence. She left me with nothing but bewilderment and despair. She obviously couldn't care less, as she made clear by refusing to answer obvious and important questions.
Meanwhile, the rash has changed in its appearance and ‘behavior’ several times over seven months. When it looked so grotesque that people averted their eyes – out of horror and/or politeness -- it caused little discomfort. As it dispersed, became less dense and more spotty, it became increasingly uncomfortable, especially at night. It flared when I was hot, upset, exerting myself, eating a warm, spicy meal and after my shower. It had ocular manifestations as well: my eyes were inflamed, gritty and exuded pus. I had wiped several floating, clear membranes from each eyeball.
I reached out again to Doug Woseth, who suggested that we do an extensive allergy test – which involved having dozens of allergens arrayed on my back for a week – and if nothing showed up, he would arrange for me to be seen at the University of Utah dermatology department’s Grand Rounds, which “is reserved for the most difficult and mystifying cases.” This was a very gracious and generous gesture on his part.
No allergic reactions occurred, so my “appearance” was scheduled for December 17.
No grandeur, but the paparazzi were nice
The prospect of Grand Rounds, which enables one to be scrutinized, assessed and discussed by some of the finest dermatologists in the state, seemed like the best chance I could have of finally learning what was happening to me. I received a notice in the mail from the U.’s dermatology clinic, alerting me to the fact that that the doctors would not be taking the time to introduce themselves or even to speak to me, which seemed as rude and intimidating as a notice I once got ordering me to appear for jury duty “under penalty of law.” I decided that – depending on my mood that day -- I would either put up with this frigid, clinical atmosphere or I’d make remarks that were so provocative, they wouldn’t be able to resist interacting with me.
Despite the coldness of the “invitation,” there was something inherently humorous in the phrase “Grand Rounds,” and I thought it would be fun to show up in a ball gown. I imagined that I would feel like both a starlet and a specimen, and that’s kind of how it turned out.
I was taken to a clinic room and told to sit on the examination table. For the first ten minutes or so, I was questioned by some very thoughtful, respectful medical students or residents. I don’t think they were very interested in me, but they did their best to come up with pertinent questions. They were so polite that they requested my permission before touching me.
Then came the paparazzi, a stampede of the Real Doctors into the small room, many of whom, according to the state’s database on public employee salaries, earn $650,000 to a million dollars annually. The cellphone cameras began flashing around me. "Turn this way, turn that way, over here please, could you toss your hair back a bit, that’s perfect, close your eyes, lift your chin, you’re doing fine!" I tried not to grin at the nuttiness of this scene – I was a serious star! They seemed like very ordinary guys, pals from a bowling league perhaps. I realized I had been silly to expect that they would fit a certain mold: tall, slender, well-dressed, serious and reserved. One was examining my scalp for hair loss – a side effect of lupus. Another scrutinized my rash, saying merely: “I’ll be damned.” One squinted at my chest, while another pressed around my ankles. They did introduce themselves, and they did ask questions, contrary to the department’s notice. Eventually, they seemed most interested in my hands, which have changed in appearance quite a bit in the past 20 years. I have attributed both the variations in color and a metamorphosis in the shape of my fingers and nails to my past history of smoking. The oxygen deprivation caused by cigarettes often manifests itself this way. It didn’t take long before the cellphones began flashing again as they took shots of my hands, while my poor, wracked facial skin became yesterday’s celebrity.
They all thanked me heartily and whooshed out the door. It was only then that I saw Dr. Woseth, who had been hidden from view, sitting there patiently in the corner. I felt as if I’d had a guardian angel with me all along, and I wished I had known. I gave him a hug – that darling guy – and went home to await the Wise Men’s assessment.
An “Unremarkable” Patient
“Don’t take it personally – it’s medical jargon,” Dr. Woseth reassured me later that afternoon. “By ‘unremarkable’ they just mean that nothing jumps out at them to explain your symptoms.” The Grand Rounds doctors’ only recommendation, he added, was that I get a CT scan of my lungs. “In very rare cases, organ cancers can have a cutaneous manifestation,” he said.
I had been wanting to get a scan of my lungs anyway. Even though I quit smoking about eight years ago, I will always be concerned about cancer. The scan came back normal.
Dr. Woseth recommended that I return to the U. for advanced allergy testing and a very refined biopsy, but I was through, at least for now, with the medical establishment. I often read about people who had to see 20 or 30 doctors before they got an accurate diagnosis, but I was too disillusioned, exhausted and pissed off for that. I would just live with this torment and be grateful that I didn’t have ALS or multiple sclerosis or a brain tumor.
It had been eight months since I had looked reasonably normal, and I had become accustomed during that time to people being taken aback by the bright-red swelling, peeling, hardening and oozing of the skin under my eyes. (Nobody would actually look at me, except for babies. They stared right into my eyes, and I really believe they felt something akin to compassion, as well as curiosity. A few even reached their arms toward me.) I wondered if I could ever grow accustomed to the excruciating, crawly discomfort and accept it stoically. People in poor countries have flies on their faces and in their eyes all the time, and they don’t pay any attention to it. So they would be my new role models for dignity and forbearance.
Fortifying My Colonial Empire
The colon is a magical place, which serves many purposes other than as a subway for excrement. Most of our immune system resides there, and most of our serotonin -- the neurotransmitter and mood modulator -- is produced there, which is one reason that the colon known as “The Second Brain.” Millions of bacteria, or maybe it’s billions (three or four pounds, I’ve read), reside in the colon. The “good” bacteria keep the “bad” ones in check. Taking probiotic supplements augments the good guys and is regarded in many parts of Europe and Asia as essential to health.
I have been taking probiotics for about 20 years, but I always got the least expensive variety, which contains only two strains of flora. At the end of January, I came across a half-price sale of a probiotic blend that contains 12 strains.
I bought a bottle, and within 36 hours, my skin was normal. Not a trace of the rash remained.
I find it hard to believe that probiotics could have “cured” me of a complex malady that had persisted for nine months. It definitely wasn’t a placebo effect, though, because it never occurred to me that the probiotics would influence the rash until I later read that dysbiosis -- an imbalance in the gut flora -- can cause autoimmune diseases as well as those I had already known about. I couldn’t think of anything else that had could have caused this radical reversal. When I told Dr. Chris Hansen about this, he emailed me: “I am willing to believe anything about your rash, because it was so unusual.” He added, “I think you are right on with the idea that in some cases the diagnosis of lupus is more of an art than a science. I reviewed your biopsy again with a few additional pathologists and I am even more convinced that lupus is not the correct diagnosis.”
But what is? Nobody knows. I think this happens in medicine more than most of us realize, and I believe that medicine is still quite primitive in many ways, despite all of its remarkable advances.
I have not been cured. My laboratory numbers still indicate that some autoimmune process is going on. I can feel the rash lurking under my skin, and it surfaces as a pink-purple shadow if I am hot or agitated or even animated. Just smoothing some moisturizer gently under my eyes turns the skin bright red for a few minutes. And at night, I can often feel the little worms in my pores getting restless, wanting to emerge. Usually, a cold washcloth is all that is required to smack them down, and usually I look and feel normal once again.
I guess I will forever be braced for another flare.