How my hands look:
How my hands feel:
“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?
|Image courtesy A Nice Ring to It|
You can’t connect the dots looking forward; only by looking back. You have to trust that the dots will connect in your future. —Steve Jobs*
When I tell people about my lupus diagnosis, I often find myself saying the word “upgraded,” as though I had been in economy class and got bumped up into first. And it always makes me laugh with more than a little black humor, because you have to be pretty sick to be excited about an “upgrade” to SLE, or any other autoimmune illness.
But the truth is that it was an enormous relief to finally have a diagnosis that included a protocol for treatment. For more than ten years I bounced from doctor to doctor, GP to rheumatologist to psychiatrist to orthopedist and back, and they all said “Well, something is wrong with you, we just don’t know what yet.” (In the meantime, I had some great therapists who helped me work through my anger and frustration and sadness, so don’t think I have anything but respect for good psychotherapy!)
As a child, it started with joint pain and inflammation, particularly in my knees and ankles. Despite my frequent fevers and general malaise, I went undiagnosed, though not untreated. Because my symptoms were systemic but primarily joint-related, I was repeatedly tested for RF, mono, and lyme disease. Over and over again. I’m old enough that at the time the diagnostic criteria for Juvenile Rheumatoid Arthritis were very narrow and required a positive rheumatoid factor (mine has always been negative)— Juvenile Idiopathic Arthritis and Juvenile Chronic Arthritis did not yet exist as possible diagnoses, and despite vascular symptoms, no one tested me for ANA until I was in my late 20s. So they pumped me full of aspirin, and I limped my way through adolescence. Periodically, as is typical in the cycle of flare and remission, my symptoms would magically lift and I would think that I had finally overcome my mystery illness, only to have my symptoms reappear weeks or months later. To call the experience discouraging is a ridiculous understatement. It was soul-crushing.
As patients, I think it’s easy for us to forget that the diseases we are diagnosed with (or not diagnosed with) are man-made entities. I don’t mean man-made in the sense of some sort of crazy conspiracy theory that involves underground bunkers or biological warfare, I simply mean that these diseases— lupus, RA, fibro, scleroderma, and many others—are relatively recent inventions in terms of human experience. These diseases have not been waiting for us since time immemorial, unchanged and universal. They each have their own history, generally one which involves an initial discovery followed by a period where a large number of people try to come to a consensus over sets of common symptoms and behaviors and the best course of action for treating those symptoms. And these sets of symptoms, diagnostic markers, and treatments change over time and we continue to learn about the mysterious (and pretty amazing) workings of the human body. The disease entities are constructed by the researchers, doctors, and patients who interact to try to understand the working of certain bodies during a certain period of time.
As an example of how the process of diagnosis can change dramatically, rheumatoid factor used to be one of the primary diagnostic criteria for RA. It is only within the last five to ten years that doctors have discovered that anti-CCP is a much more useful test for diagnosing and treating RA, particularly in its early stages. And this was not officially written into the American College of Rheumatology diagnostic criteria until 2010! Unfortunately, even as medical researchers begin to understand the mechanisms of autoimmune disease better, and treat the underlying causes—for example with biologics that target specific immune cells—rheumatic and connective tissue diseases bring with them a whole host of stereotypes and negative connotations.
I’ll save my diatribe on women and autoimmune illness for a later blog post (remind me to tell the story about the orthopedist who told me I needed more brisk walks…), but I just want to end by saying that I think it’s important to remember that you are not your illness. Neither you nor the illness you have been diagnosed with is a solid, frozen, unchanging entity. Flares will come and go, symptoms will come and go. Your own self identity will change during the course of your life. Your sense of self and your identity have been shaped by your past experiences and the culture you were born into and currently live in. In much the same way, the illness you have been diagnosed with is shaped by the cultural and scientific world(s) in which you live. The very definition of your disease may change in your lifetime. Don’t let it rule you—acknowledge it, seek to treat it in ways that allow you to live a richer life, but remember that you are an active participant in your own becoming.
*Quote courtesy ZenMoments‘ Twitter feed.