It’s been nearly four months since I last blogged. I suppose that means I’ve been on a bit of a hiatus, partly intentional, partly unintentional. My personal life imploded in April, and while I won’t go into it here, I will say that some of the issues have been resolved, and some haven’t. I am trying to be at peace with uncertainty.
The sense of uncertainty extends to my health as well. I had a good five month stretch of prednisone-enabled denial, where I was nearly symptom free. It was lovely, but obviously too good to be true, and about a month ago my symptoms started to emerge again. My rheumy had hoped that a few months of continuous steroids would allow the additional Plaquenil/HCQ to take effect, and then we could taper down again. Unfortunately, it hasn’t quite worked like that, and I find myself with considerable pain and fatigue, despite being on the highest dose of HCQ for my weight, and a maintenance dose of steroids plus massive amounts of ibuprofen (and everything else…). So we’re onto the next DMARD, Arava. Or trying to be. My insurance has denied coverage for it, even in generic form, so I’ve spent the last three weeks battling that and also applying for medication assistance through Rx Outreach.
Back in June I gave a talk on children’s literature and illness narratives at the Society for Disability Studies conference in Denver. It was my first year attending, and I have to say that the conference itself was an extraordinary and often joyful experience, though not without some feelings of anger and frustration. I think in some ways it is even harder to have an invisible illness or disability among people with visible and/or mobility-related disabilities. The scrutiny is much higher, and some people can be much quicker to judge or assume that one is able-bodied. I felt significant pressure to “explain myself” and my presence at the conference. I’ll have to write more about the experience in another post.
“The wolf, I’m afraid, is inside tearing up the place.” —Flannery O’Connor (from a 1964 letter)
So after about five magical weeks of feeling like my functional pre-MTX (but not necessarily pre-lupus) self, the fatigue has descended again. I’m sure the time change is part of it, but I could feel the creep of the fatigue all last week. I wrote it off as lack of sleep and excitement/stress from starting my new job, but now the gnawing has started up in my wrists, and I know it’s the inflammation stirring again. (Wren calls the pain of RA her “rheuma-dragon,” but I think of my joint pain as the arthritis wolf. I’m sure that’s partly the power of suggestion—”lupus” = “wolf”— but it’s just such a gnawing pain that I can’t imagine it being anything else. And we still don’t know for sure whether it’s lupus, RA, or both.)It’s hard to give myself permission to be tired, to say to myself: “Rest! Your body is telling you that you need to take care of yourself!” rather than to chide myself for being lazy. Because it’s always possible to push a little further, force myself to go a little bit longer, forego more sleep than is comfortable (carry one more load of laundry or recycling up and down the stairs…). But eventually I crash, and then what do I have? Well, I morph from chronically ill to acutely and chronically ill. And that helps no one.At my last appointment with my rheumy she asked me to try to wean myself off my ibuprofen habit, and we also increased my Plaquenil dose to 600mg (visual field test here I come). I tried—and succeeded!—to reduce the ibuprofen, but in the past couple of days I’ve been in the kind of pain that just laughs in the face of Tylenol and Tramadol, so I’ve had to undo all the careful calibrations I made last week. Back on the NSAID train. One step forward, two steps back.Now where have I put all my spoons? I need one for my writing, one for my Wednesday swim, one for each day in the office, and about fifteen for the friends who are coming to stay with me this weekend…
I’m not lazy, I’m sick. And I’m doing the very best that I can. It feels pretty heroic from my vantage point.
“But the beginning of things, of a world especially, is necessarily vague, tangled, chaotic, and exceedingly disturbing. How few of us ever emerge from such beginning! How many souls perish in its tumult!”
Sometimes you think you have everything figured out. You’ve found the key that unlocks the door and everything will be revealed. Except life so rarely works like that. Every ending is a beginning. Every solution is simply the beginning of another question. The wheel of fortune just keeps spinning. And I don’t mean the one that involves earning money by spelling words correctly, I mean the one that came before, literally “Fortuna’s Wheel,” depicted to the left. Welcome to the spinning wheel of life with autoimmune disease, where no spot on the wheel will get you a new car or a vacation to Hawaii, but it might get you back to where you started in the first place.
Several weeks ago I wrote about my most recent visit to the rheumatologist. He reviewed my bloodwork and asked how I was feeling. I had to answer honestly that my joints were killing me, especially my hands + wrists and feet + ankles. He took a look at them and said we needed to do more, that the Plaquenil was helping my other symptoms, but that if I was relying on the ibuprofen for my joints, I was just masking the inflammation and not altering the course of “the disease.” Of course, the problem in my case is that we don’t know exactly which disease entity we’re working with. (Still. Yet. Again. Have I mentioned I have trouble with labels? I have trouble with labels.) So “the disease” can’t really be pinned down. My symptoms (vascular and skin-related) last winter and my initial response to Plaquenil (also called hydroxychloroquine or HCQ) and prednisone, plus my negative Rheumatoid Factor (RF) suggested lupus. But after seeing the sad state of my joints as of a month ago, rheumatoid arthritis is back on the table. Are we looking at rhupus? MCTD? One of the spondylopathies? I still don’t know.
First, a little history. My understanding is that my grandmother—who died in 2002— had some form of what might now be diagnosed as Mixed Connective Tissue Disease (MCTD). She was originally diagnosed with “arthritis,” though how long ago, I’m not sure. Later, scleroderma was added, and I’m honestly not sure if there were other autoimmune issues at play. The scleroderma presented primarily as CREST, affecting her skin, GI system, and causing Raynaud’s. As a child, we were very close and I always loved going over to her house. Some nights I would sleep over so my parents could go out, and she and I would stay up late, playing cards and eating cookies.
She had all sorts of wonderful “devices” in her house that made things easier, even things like playing cards. There was an automatic card shuffler and wooden boards with horizontal grooves in them to hold a handful of cards without showing them to your opponent. She also had Tupperware that you could seal by pressing the center of the lid with your elbow and little squishy rubber things to make gripping a pencil easier. Not realizing that she had autoimmune disease, and not your standard run-of-the-mill Osteoarthritis, and that she probably couldn’t have shuffled a deck of cards or closed a Tupperware lid with her fingers alone, I just assumed these were fun gadgets. After all, I sometimes had trouble getting Tupperware containers to “seal,” and shuffling cards was endlessly complex for my 6- or 7-year-old-hands. I even assumed her hands, disfigured by the classic signs of inflammatory arthritis—ulnar drift, boutonniere deformities, swans necks—and the shiny skin of scleroderma, were what the hands of all “old people” eventually looked like. My father even tried to scare me out of cracking my knuckles by telling me I’d end up with hands like my grandmother’s. I know now that this is impossible, and yet I—so vain about my long slender fingers and pale skin—may end up with her hands regardless.
I don’t think anyone expected my grandmother to live as long as she did. I’m not sure they thought she would live past her 60s, and yet she lived well into her 80s. But the effects of her disease(s) and treatments took a terrible toll on her body. Everything from her teeth to her skin to her esophagus to her kidneys (and who knows what else) were slowly destroyed. It was only when I was older that I really began to understand all that she’d been through, working full time as a nurse while my grandfather fought in WWII. And that she’d continued to work after the war, mostly for economic reasons, but still an unusual choice during that era. I wish I’d been able to ask her about her diagnosis, the pain she dealt with, how her doctors treated her. Everyone says we like alike, in looks and in our stubbornness and tenacity. People have even assumed that photos from her teens and 20s are actually black and white photos of me. I just never realized until now how alike we really are. And I wish I still had her here. Because I’m scared, and I need her to assure me that I’m going to be OK, that life goes on, and that it can still be rich and beautiful and fulfilling and full of love, no matter which box gets checked, no matter what diagnosis code is written next to my name.
This entry was featured on the Patients for a Moment Blog Carnival: Revenge of the Conquered, or is it Avenge of the Conquered?
“I can with one eye squinted take it all as a blessing.” –Flannery O’Connor
After I posted the photo of Flannery O’Connor yesterday, I went back and read more of her letters and thought about what it must have been like to live through Georgia summers, with lupus, in the 1950s and 60s. More than that, though, I thought about what it must have meant to be diagnosed with systemic lupus erythematosus back then—back when your only options for treatment were corticosteroids and aspirin, back when it took so long to get a diagnosis and the treatments were so limited that it was basically a death sentence.
In the 1960s, scientists discovered that the anti-malarial drug Plaquenil was effective in both rheumatoid arthritis and SLE. It has been one of the only drugs approved for the treatment of lupus. Those of you playing along at home may be laughing, sardonically, since the treatment protocol for lupus has changed little since the ’60s, just swap out the aspirin for ibuprofen or naproxen and add a dash of low-dose antidepressants for the co-incident fibromyalgia.
Given this history, then, it’s no surprise that most of us were thrilled to hear that Benlysta, heralded as the “first new lupus drug in 56 years!” was approved by the FDA this past March. Benlysta (belimumab) is a medication delivered by IV that targets B-cells, a part of the immune response that seems to be out of whack in lupus patients. (The science is significantly more complex than that, involving an intricate dance of activators, antigens, modulators and interactors. If you like that sort of thing, look here for a PubMed citation.)
But the emergence of Benlysta on the scene isn’t exactly a magic cure-all. In fact, the FDA vote was extremely close because of concerns that the drug was only marginally effective. This short piece from the Associated Press somehow manages to capture the breathless excitement and serious concerns about the drug all at once, both of which also come through in a slight muted form in the FDA press release. Among the serious concerns: the drug appears to be ineffective in many patients of African descent (who, by the way, tend to be diagnosed with lupus in much higher numbers along with other non-white populations). What the press release and other news coverage also frequently fail to mention is the exorbitant price tag for Benlysta and similar biologic drugs used for rheumatoid arthritis and other conditions. Like thousands-of-dollars-every-month expensive. Like don’t-expect-your-lousy-hmo-insurance-or-medicaid-to-cover-it expensive.
Luckily, scientists are still hard at work to map the far corners of our immune systems, dysfunctional or not. The most recent issue of The Rheumatologist includes an update on research into the role of T-cells in lupus that could lead to more effective treatments for more people. And I’m excited about that. What I’m also excited about is my chance to participate in the American College of Rheumatology’s “Advocates for Arthritis” Program. When I go to Washington this fall to meet with members of Congress about the pressing need for research funding for arthritis, I will cite these studies, and the studies that led to the development of Benlysta, and their importance in improving the everyday lives as well as the long-term prognosis for people with lupus. Fifty-six years is not ok, people!
O’Connor, diagnosed at age 25 in 1951, outlived the five years that her doctors gave her with an additional nine, making it almost to her 40th birthday. I can only imagine the pain and fatigue that she worked through during those years while she managed to complete two novels and several collections of short stories, even as her body began to fail. Most of us now, diagnosed with Lupus in our 20s and 30s, have a much better chance of having a “normal” lifespan, simply because there are more ways to treat the complications from lupus. We’ve gotten better at catching kidney, lung, and heart involvement earlier and addressing these issues and slowing their progression before they become life threatening. We know about the Faustian bargain of long term use of prednisone and other corticosteroids. And I hope—with active and public communities on Facebook, Twitter, and other social networking sites (hi, chronic babe! hi, but you don’t look sick!)— we are getting better at shedding the stigma of having a chronic illness and speaking out about what we need. The magic of the internet lets us all be activists, even in our pajamas. And that is really something to be excited about.
Edited to add: This entry was featured on chronicbabe.com in Blog Carnival #34: What’s HOT?