Wednesday, June 3, 2015

Lupus Update: I surrendered

    (Jan. 14, 2012)  I have gotten a large and ongoing response to my posts on lupus, particularly the ones in which I explain  my decision not to take prescribed medications. I wrote that I intended to rely on probiotics, my vegan diet and daily exercise to keep myself as healthy as possible.
    For more than a year, I ignored the advice -- and in one case the pleas -- of four doctors, who believed I should be taking the anti-malarial drug Plaquenil.
    In November, I finally relented, when some new and distressing symptoms emerged.

Washington, DC (PRWEB) October 25, 2013

International knowledge healthcare provider BMJ and the Lupus Foundation of America (LFA) have announced that they have joined forces to publish the first open access journal dedicated to the poorly understood and potentially life threatening disease, lupus. Lupus Science & Medicine will offer timely global access to the latest scientific advances in lupus. The title will officially launch early next year.

    I have still not received a credible, positive diagnosis for my autoimmune condition. It seemed crazy to me to inflict a medication on my body without knowing what is wrong with me, especially when neither the medical profession nor the pharmaceutical industry understands how Plaquenil mitigates lupus -- or even what it does to your body, as a whole, after you swallow it.
    Two male doctors pressed me to try it anyway, saying, "If you were my mother, I'd beg you to take it."
    (Nobody ever says "If you were my daughter" anymore, or even, "If you were my sister." Suddenly, I'm everyone's mother. That wasn't my plan.)
    The female rheumatologist I saw said, "Maybe the drug will help you feel better," even though she had just said I no longer met the criteria for a diagnosis of lupus.

    Why is there no explanation for how and why this drug might make me feel better? What would it do? I'm not accustomed to conducting my life in an uninformed way. I'm in the dark, and it seemed that she was as well.
    An internist reasoned, more convincingly, that I had a lot to lose by not taking the drug, because of the unseen damage my autoimmunity could be doing to my internal organs. 
    Still, I pressed on, drug-free.
It's disheartening to feel so ill. Is it all "in your head"? No!
    Then, months ago, my facial flushing evolved into a striking malar formation. The fiery rash I had for nine months last year was not malar. I now develop this new configuration each day when I take my shower, when I dry my hair, when I take a nap and when I do stretching that involves bending from the waist. Jogging on a treadmill (when the air quality outside is poor) leaves me with a dramatic purple "butterfly." When I have an animated or stressful phone conversation, out it pops. When I eat a hot or spicy meal, the skin under my eyes itches and burns. 
    My overall facial flushing has worsened, but it is the malar aspect that troubles me most, since that is essentially a "calling card" for lupus.
    More recently, my vision problems have increased, my typing is ridiculous -- I constantly make typos while trying to correct typos -- and my balance has become more impaired. I have also developed pernicious anemia and an unusual abdominal pain. My hair is thinning and breaking off -- very unattractive. My nose is becoming BIGGER (and I'm not lying!) from repeated inflammatory episodes. It's a good thing that my looks aren't very important to me anymore. I would really be devastated about what is happening to me.
    Despite these new symptoms, I continued to reject medication. 
    The last straw came when I began stuttering quite badly. This really disturbed me, because it suggested that the disease might be attacking my brain. I checked out some message boards and found that many women with lupus develop a stutter, and some also acquire a sort of "mind stutter," which causes their thoughts to race ahead of their ability to grasp or vocalize them.
    During one phone interview, my speech became so excruciating that I ended the conversation apologetically, saying, "We're going to have to do this via email."
    That was enough for me. I began taking 600 mg. of Plaquenil about 10 weeks ago, well over a year after it was initially prescribed, while continuing with my nutritional, exercise and probiotic regimen.

     I also take black currant oil capsules, which are recommended by Dr. Andrew Weil. They, and borage oil, are good sources of gamma-linolenic acid (GLA), and I believe they have mitigated my overall feeling of unwellness.
    (See "Lupus, the blushing terrorist, bulges with cruel surprises," for more: ).
    My symptoms continue to be unpredictable and very stressful. But I'm glad I'm taking Plaquenil. New data in 2013 indicate that the drug moderates flares, reduces organ damage and acts as an anti-inflammatory.

    There are times, though, when I do have an overall sense of simply feeling better, as the rheumatologist indicated I might. For years, I have felt ill most of the time, which I have attributed to major depression. Now, there are hours at a time when I feel more comfortable and even quite normal. But there are also days when I am as wracked and wiped-out as ever. So I don't know if the Plaquenil has anything to do with the episodes of well-being. If it did, wouldn't it be more consistent? And wouldn't it be modulating my more conventional lupus symptoms, such as the rash?
    Lately, I have noticed that my posture is deteriorating at an alarming rate. I had never heard that lupus accelerates the loss of bone density, but according to the National Institutes of Health, "Studies have found an increase in bone loss and fracture in individuals with SLE. In fact, women with lupus may be nearly five times more likely than those without the disease to experience a fracture from osteoporosis. Individuals with lupus are at increased risk for osteoporosis for many reasons. To begin with, the glucocorticoid medications often prescribed to treat SLE can trigger significant bone loss. In addition, pain and fatigue caused by the disease can result in inactivity, further increasing osteoporosis risk. Studies also show that bone loss in lupus may occur as a direct result of the disease."
A growing number of cases involving chronic sinusitis is being reported.
     Then I thought, what the hell, lupus seems to cause or exacerbate everything I have. What about sinus problems? Voila! According to the winter 2007 issue Musculoskeletal Report journal,  "If a lupus patient has  chronic sinusitis or some suggestion of immunodeficiency, you should be tested for immunoglubulin levels." This is the conclusion of  lupus specialist, Dr. Robert Lahita, the editor of Systemic Lupus Erythematosus.
    "Systemic lupus erythematosus (SLE) and common variable immunodeficiency (CVID) are at opposite poles, immunologically speaking, but a growing number of CVID cases is being reported in lupus patients. Because SLE develops first, rheumatologists should monitor immunoglobulin profiles in lupus patients and should consider CVID in all lupus patients who have recurrent sinopulmonary infections in the absence of lupus activity and/or immunosuppressive treatment,"  according to a study in the February 2007 issue of Seminars in Arthritis & Rheumatism.

This spray works wonders for pain and pressure.
     I had suffered for years from chronic sinus inflammation and discomfort for years, but I have gotten considerable relief from daily steam inhalation ($20 product from Walgreen's), oil of oregano capsules (which have antibacterial, antimicrobial and antifungal properties), and Sinu Orega, a nasal spray containing oregano, cloves, sage and bay oils in a saline solution.  
    So I thought: Maybe lupus could be implicated in my exhausting and bewildering increase in dental problems. Doesn't that sound pretty far-fetched? 
    Not at all, according to Dr. Michael Huber, in a Lupus Foundation Forum.
This, plus bald spots and a hunch back, make you feel like hiding under the bed.
   "Reduced salivary flow increases one’s risk of a variety of oral maladies such as oral infection (cavities, gum disease, fungal infection), along with difficulty with speaking, eating, and swallowing," he said.  
    I'm going to give the medication a while longer to see what happens. Some sources say it can require six months to take full effect. 
    The first doctor I saw, about the fiery, raised rash under my eyes, declared -- as soon as he walked in the room -- "I know what you have! You have lupus!"
    I had come to see Dr. Brian Williams, a Salt Lake City dermatologist, after trying to treat the rash myself for four months.
    "I'm not going to take steroids," I told him.
    “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.”
     I felt even then that he was just trying to upset me, for some sadistic reason. Anti-malarials such as Plaquenil seem to have fewer side effects than most drugs. My stomach is upset, which is common. I haven't had my eyes checked yet, but retinal damage wouldn't have appeared this early anyway, and from what I have read, the danger of retinal toxicity is quite minor.
Retinal photograph showing classic “bull’s eye” retinopathy of Plaquenil toxicity,
     According to the National Data Bank for Rheumatic Diseases, the risk is below one percent for toxicity within the first five to seven years of Plaquenil treatment, as long as dosage is within general guidelines (800 mg. per day or less for those of normal stature). Those with short stature (5' 2") should not exceed 400 mg. per day, according to Michael Marmour, in The Rheumatologist journal of May 2011.
    "However, frequency of toxicity rises thereafter, probably reaching several percentage points after 15 to 20 years of use," the study adds, although even that is a relatively minor risk. Another study found that the risk for retinal damage is less than one percent, even at doses of up to 1,000 mg. per day, and even after long duration.
    Regular eye exams are still recommended, especially for those who have been taking Plaquenil for several years. A baseline retinal exam is advised within the first year after a patient starts the drug to assess any underlying maculopathy. Dr. Marmour strongly advocates seeing an ophthalmologist who has access to high-resolution, spectral-domain optical coherence tomography (SD-OCT), which is much more sensitive than older technologies in discerning retinal changes.
    Plaquenil also makes patients more prone to eye infections, such as conjunctivitis, which can be treated with topical antibiotics.
     In spite of the confusion and anxiety I feel about having whatever it is I have, I just keep reminding myself to be grateful that I don't have something worse, and there are so many things that are.
    Winter 2014-Spring 2015: the rash returns, along with massively debilitating weakness and vertigo:
    I have never received any benefit from "medical care" with respect to my condition. Doctors charge me hundreds of dollars in exchange for recommending exercise and a good diet (which I have done to death for years) and stress reduction, which I am innately unable to achieve. My questions about autoimmunity appear to have no answers, and the expensive eczema creams (example: $140 for a tube of Protopic) provide no relief whatsoever in treating the discomfort or in making the rash go away.



    Why is it so hard to get answers to basic questions about autoimmunity? I have raised the issues below with several highly regarded experts nationwide, and I have gotten virtually no useful information.  I spoke to a professor of immunology who said, "The questions you pose are the very questions we are trying to answer. You have actually painted quite a clear picture of this complex, baffling disorder just by framing it as you have."
    I don't buy this. I am starting to wonder if they have any idea what they're doing when they "treat" us. How can we trust either their diagnosis or their "medical advice" if these fundamental issues remain a "mystery"?

    At the bottom are some insights sent to me by Dr. Noel Rose, director of the Center on Autoimmune Disease Research at Johns Hopkins University.  I am still hoping we can get additional information on these issues:
   1.  Once autoimmunity has been set in motion, does it ever go away? Should we assume that we will endure this affliction forever?

    2. Can a healthy, active lifestyle (or any other intervention, or pure luck) enable the body to “cure” itself? Have meditation, yoga, acupuncture or other alternative therapies ever been shown to be effective in returning the immune system to normal? Can they lessen the degree of autoimmunity?

    3. Is autoimmunity progressive, i.e., should we assume that our ANA and other numbers will  rise, or are they likely to remain at the elevated levels that were initially discovered? Should we be retested periodically to assess our numbers, or is this of no value?   

    4. Will taking Plaquenil alter our lab tests for ANA, etc? Can it actually lessen our degree of autoimmunity?

    5. When lab results indicate that autoimmunity is present, how much difference do the numbers make? If ANAs, for example, are elevated to a relatively low degree, what should our course of action be?
    At what level does autoimmunity become a disease state?
    Is damage occurring within us even at low levels and even if doctors can‘t attach a name to the “disease”?

    6. If our lab work indicates autoimmunity, but we are unable to obtain a specific diagnosis, what should we do?

    7. Do higher numbers indicate that a process of greater severity and with greater potential for damage is occurring?

    8. In other words, are there degrees of autoimmunity? How does treatment differ with varying degrees? Should one’s decision on whether to take medication be determined by the lab numbers that depict the level of autoimmunity?

    9. I have read that the chronic inflammation which is associated with lupus and other autoimmune conditions can affect the skin, heart, lungs, kidneys, joints, and nervous system. My C-reactive protein tests indicates that I am free of inflammation. None of my doctors asked about this or tested me for it, even the ones who said I have systemic lupus -- I had the test done at my own initiative. How can this apparent discrepancy be explained: autoimmunity but no inflammation?

    10. Does the damage occur to us internally only during flares, or is it happening all the time like it is with high blood pressure? Is there really no way to tell if we are having an internal flare?

    11. What is a flare, exactly? What occurs physiologically during a flare? How long do they last? How is it that they can affect isolated organs when autoimmunity is systemic? Does each flare affect the same organ, or can they 'move around'?
    12. Is someone who has laboratory findings consistent with lupus, but who has not been conclusively or convincingly diagnosed with lupus by any of several doctors, still vulnerable to the internal “flares” and organ damage that a lupus patient faces?

    13. Should we be getting regular tests to monitor kidney function once autoimmunity has been discovered? What other tests should be considered to monitor possible internal damage due to autoimmunity? Are there tests to monitor possible neurological damage?

    14. Do ups and downs in lab results for immune function indicate that our condition is changing, or is the variability irrelevant to the seriousness of our condition?

    15. What factors might contribute to improvement or deterioration in the severity of our autoimmune situation?

    16. When one has elevated ANAs and other immune-system indicators but doesn’t have a specific diagnosis, what does that imply about our current state of health, and our future? What is the prudent action to take?

    17. Do elevated antibody readings mean that we have an autoimmune disease of some sort, even if it’s not lupus? If not, what does it mean? Does everyone fit into one of the 80 to 100 autoimmune disease categories, or do some of us have a “nonspecific” autoimmune condition?

    18. Is there some general regimen that a person with a nonspecific or undiagnosed autoimmune disorder should follow?

    19. Does the patient with lupus-like autoimmune lab results,  but who is said not to have lupus, need to take the extreme precautions regarding UV exposure as someone who has been positively diagnosed with lupus?

    20. Since I exhibited enough of the criteria in one month to be diagnosed with lupus but not enough two months later (although the lab results remained the same), is it possible that in subsequent months, I will once again face the prospect of a “definitive” lupus diagnosis? And then another reversal?

    21. Some of us have lab results that refer to a “high chromatin antibody level,” which is described as a frequent "early marker" of systemic lupus, yet doctors have not attached the name "lupus" to our condition. What are the other meanings that “high chromatin antibody level” can have? In what way should we follow up on these readings. as well as the DsDNA readings?

    The data I have seen indicate that high anti-chromatin antibodies virtually always indicate systemic lupus and are never found in healthy individuals.  According to the NIH, anti-chromatin antibodies are both sensitive and specific for SLE, and are a useful marker for an increased risk of lupus nephritis. "Anti-chromatin antibodies are not generally found in any other disease, thus showing very good sensitivity and specificity for patients with lupus,"says an article in the Annals of the Rheumatic Diseases. So on what basis would a doctor ignore these readings and retract a lupus diagnosis?

    22. When one has an autoimmune disorder, how does it affect the immune system’s ability to fend off the external invaders that it was intended to fight? If our immune system is engaged in attacking us, can it still protect us from the flu, pneumonia, shingles, cancer, etc? Is it safe to get a shingles vaccine?
    When one has an autoimmune disorder, are things that are harmful in general more harmful, such as pesticides, volatile organic compounds, pollution, sugar, chlorinated and fluoridated water, mercury fillings, allergens, etc.?

     23. If our lab testing indicates a low red and white blood cell counts and a high MCH and MCV, and if our blood-oxygen level drops significantly, could that be explained by autoimmunity? Are there treatments for this condition? Would B12 shots be indicated?

    24. Is it true that Vitamin D exacerbates autoimmune disorders, as Dr. Trevor Marshall has so intriguingly argued?

      25. If testing indicates that autoimmunity is present, is our immune system attacking us, even if doctors are unable to attach a name to our autoimmune condition?

    26. What does it mean if a patient who has always had ANA numbers within normal range develops a persistent increase in (positive reading) ANA Screen IFA, ANA TITER ANA, out of range double-strand DNA, a nucleolar pattern and an increasingly high (out of range) result on the antichromatin antibodies?
    It does seem that something which is NOT GOOD is happening in the body. Is that not the case?

    27. Is a 1:160 ANA titer what you would regard as a 'low level'? The lab numbers range from negative to low to elevated, and "elevated" is designated at 1:80. So is 1:160 'low level'?

   Dozens of my readers have reported this situation: Lab tests indicating an autoimmune issue plus the presence of many diagnostic criteria. Some doctors say they have a disease, others say they don't, some -- as in my case -- saying I do and two months later saying I don't.  Meanwhile we're warned about irreversible organ damage, even as we're told we don't seem to meet "enough" of the criteria "currently." One doctor acknowledged to me that diagnosing lupus is more art than science, but it's seeming more like a game of craps.

    28. LIKE MOST OF THE WOMEN WHO HAVE WRITTEN TO ME with elevated antibodies and other abnormal blood work, I feel that I am ill.  Are we all just neurotic?

    29. Do you believe that we should take Plaquenil "just in case," when various doctors equivocate on our diagnosis?

    30. Do you believe that probiotics can play a role in modulating autoimmune processes?

    31. If elevated ANAs are of no concern without "clinical parameters," as Dr. Rose indicates, what causes them to rise?
   32. If autoimmune diseases "wax and wane," does the internal damage that they are causing wax and wane as well? If your rash fades, for example, or if some of your other criteria improve, can you consider yourself "well" for the time being, or at least less unwell?

    Dr. Noel Rose, director of the Center on Autoimmune Disease Research at Johns Hopkins University, has been publishing articles on this subject since the mid-1950s.
    In his correspondence with me, he provided the interesting insight that, "Autoimmunity is not disease.  Autoimmune disease is a clinical diagnosis, and laboratory tests serve predominantly as confirmation."
     Low levels of anti-nuclear antibodies (ANA) are very common and are rarely a cause of concern in and of themselves, he adds.   "The presence of a positive ANA or other laboratory determination is by itself not indicative of disease.  It may remain the same, may disappear or may predict later disease. If there is no clinical indication, there is no reason to repeat such a test. "
     Dr. Rose stresses that laboratory tests are secondary. "Autoimmune disease is diagnosed by history and clinical signs and symptoms," not by numbers, he says. If you don't display the outward signs of lupus, for example, you don't have lupus -- no matter what your ANAs, anti-chromatin antibodies, CBC, etc.
     For patients who are concerned about the amount of damage that is being done to them internally by an autoimmune condition, the laboratory numbers are just one factor that is considered, he says. "This is a functional determination and not necessarily related to the amount of antibody," he adds.  
    Your treatment should be determined by your symptoms, not by your blood work, Dr. Rose says.  He also states that autoimmunity doesn't necessarily increase inflammation, so a low C-reactive protein test doesn't mean that an autoimmune process is not in effect.
    Your lab results can vary over time without an actual change in your clinical condition, he says.
    "Autoimmunity is by its nature specific, but the disease may not be well localized particularly in the early stages," Dr. Rose explains.
    Most autoimmune patients do not lose their normal immune response, he says, and are thereby not more susceptible to getting sick. However, some of the drugs used to treat some autoimmune disorders may impair protective immunity, he adds. 
    "Some forms of anemia such as hemolytic anemia or pernicious anemia may be related to autoimmunity," Dr Rose says.