Here We Go Again

September 5, 2015 at 12:44 pm (Uncategorized) (, , )

Brace yourself. I’m about to do what I’ve done for so many of my past blog entries—apologize for being gone so long from this written world, explain whatever misfortune has recently befallen me because of the rotten chronic illness I’ve got, and vow to do better as a blogger.

So here we go. Again. I am sorry for being absent from this blog. But unfortunately I’ve been having quite a time of it. Starting in July, my sarcoidosis began to flare. The pain in my head quadrupled. I wasn’t able to get out of bed. To make things worse, a combination of the neurosarcoidosis in a particularly non-sexy manifestation called diabetes insipidus along with the side effects of a medication to control this weird symptom of weird disease caused my blood sodium levels to drop to dangerous lows. Who knew that sodium is necessary for proper cardiac and neurologic function? I sure didn’t, but I learned quickly, when my heart began racing so wildly that my defibrillator kicked in and paced my heart for nearly 48 hours straight.

I couldn’t cope at home. So I wound up in the hospital, for 33 long days and nights. They took very good care of me. My doctor and his team of nurses are truly extraordinary, both in their skills and their kindness. But, still, it was the hospital. I was hooked up to a pain medicine pump. I was poked and prodded, given intravenous steroids that made my moods erratic and mysterious. Even the intravenous drip from the pump couldn’t contain the exploding pain in my head. I’m sick of calling this particular affliction a headache. It so minimizes the catastrophe in my head—and the catastrophe it wreaks on my family’s life. (I just might have a contest to see who can come up with a better, more severe sounding word than headache for something that lands me in the hospital for over a month.) There were bright spots, of course. My sister came out for a week to help Jay and Andrew and me make it through this ordeal. So did my parents. Overall, the month was bleak. But I improved enough to make it home, and now I’m slowly regaining a little bit of all the strength and stamina I lost by laying flat on my back for 33 days. It’s millimeters, not miles, a day.

The problem with landing in the hospital for a month or two every year or two (or a couple of times a year, depending on the disease’s fickleness), is that it becomes routine for everybody else. Except me. At least 75 percent of the nurses remembered me. This was nice, but it made the whole process feel disarmingly familiar. Jay and Andrew immediately went into crisis mode. Jay figured out a way to sleep at home with Andrew and make it to the hospital by 5:30 AM for the doctor’s morning rounds. It involved sleep deprivation. Jay and Andrew exhausted our town’s to-go options and we ate dinner as a family in my room most nights. Jay texted all my close friends updates about my condition and I got lovely responses, which all included some version of, “It really sucks you are back there.” But my terrifying pain, my loneliness, my anxiety that I would never get out, never be able to get away from the rank beast of pain crouched in the corner of my brain, felt all new to me.

Coming home was hard. For a week, I literally could not stop crying when I spoke to people. Their kindness, especially, did me in. I had the shakes for a good while too, and I seemed to have picked up a nervous tremor that I cannot yet ditch. I had to face Jay’s exhaustion and Andrew’s pain at losing his mother for a month with no warning and no guarantee that it won’t happen again.

So while in one sense, I could plug this blog post into my familiar template, and I joke about “here we go again,” in another sense, this is all terribly wrong. This trauma was unique. It may fit the narrative flow to classify it as “another one of Rebecca’s long hospitalizations.” But for me, it was a month of pain, my month of pain, which was like nothing that came before it.

I’m tempted nonetheless to follow my old format and promise you that I’ll be back here, writing X days a week. But I won’t. I can’t. I am creeping back to a life not spent entirely in bed. I only cry occasionally now. But Jay, Andrew, and I are not back to normal. We are carving out a new place for ourselves in Chronic Town. I’ll write again when I can, and I’d love to hear from you. Keep me in your thoughts, please, as I try to figure out what recovery looks like.

So, what would be a better word for headache? Can you think of any word or phrase that does a better job encapsulating the severity and duration of mine?

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Caveats

February 6, 2015 at 4:08 pm (Uncategorized) (, , , , , )

Pardon my long absence. I’ve had an eventful—and at times grueling—few months. But I’m finding my way back to writing, and hope to post more regularly here.

If there’s a theme to my news it’s this: I’ve gotten a lot of good news, but good news with caveats.

I’ll talk about my other news (like a more involved knee surgery than I thought I’d signed up for and medication side effects) in future posts, but let’s start today with my sarcoidosis. In December, I went to Ohio to meet with my sarcoidosis guru to assess the impact Acthar is having on my systemic disease. Acthar is the new treatment I was able to start on last May after the National Organization for Rare Diseases (NORD) was generous enough to enroll me in their patient assistance program after my insurance company denied coverage for this very old but newly very expensive drug. I was desperate when I began the twice weekly home injections of Acthar, stuck as I then was on an unsustainable regimen of toxic drugs that seemed barely to control my sarcoidosis—two monthly chemotherapy infusions (Cytoxan and Rituxan), one chemotherapy pill (CellCept), and prednisone. And during the past seven years, I had “failed” an even longer list of equally toxic drugs that did even less to stop the disease’s victory march through my body than the ones I was on. Acthar felt like my last hope.

I went to Ohio bearing films of whole body PET scans done before I started Acthar and six months after the drug had a chance to begin working. The comparison, according to my guru, was “astounding.” Acthar diminished the active sarcoidosis in my body by 50 percent. I need to repeat that because the news still takes my breath away. In six months, Acthar did what a host of immunosuppressants, chemotherapies, TNF inhibitors, biologics, and corticosteroids could not. It not only stopped the sarcoidosis from progressing, but beat it back. This is great news in itself, but it also means I can stop both Cytoxan and Rituxan. I can look forward to fewer nasty chemo side effects, and hopefully more energy as I quit these toxic brews.

There is, however, a caveat. As much as Acthar is demonstrably working to fight my sarcoidosis, I am not feeling much better. I continue to struggle with almost daily migraine-level headaches, along with frequent bouts of vertigo that are so intense I can’t get out of bed. I’m still exhausted every day, even after sometimes sleeping fourteen hours a night. The left side of my face is still numb, and my left leg is so numb that after my recent knee surgery, I gave myself second degree ice burns without feeling a thing.

“What is going on?” I asked the sarcoidosis guru. “Why don’t the encouraging test results mean that I feel better?” It turns out that the disease has damaged—probably permanently—the delicate cranial nerves that are responsible for my pain, vertigo, and vision problems. My chronic fatigue and various areas of numbness are also caused by nerve damage. There is no current treatment for peripheral neuropathy.

If I plotted my emotions during my hour visit with the guru and in the weeks since then the result would look a lot like a sine curve. My hopes rise, dip, rise, and dip again, and I end up pretty much back where I began. My inner dialogue goes something like this:

“I’m so much better! Acthar is actually working”
“But my head is throbbing and my world is literally tilting.”
“I get to stop Cytoxan and Rituxan! That will help me feel better, maybe a little less tired.”
“Who cares? I have permanent nerve damage and there’s no hope for improvement.”
“But I’m so much better. Acthar is actually working.”
(Repeat. Ad infinitum.)

It’s exhausting to be on a rollercoaster of emotions. In turns, I am elated, deflated, hopeful, and gloomy. I push myself to appreciate the truly good news I received. I catch myself wallowing in the caveat. When I inject myself twice a week with Acthar, half of the time the syringe feels like it contains a golden elixir of hope itself. “This will cure me,” I think. But the next time I’ve got to plunge a needle into the muscles of my thigh, it only hurts and makes my hands shake. What does it even mean to be cured if the damage the disease wrought means I’ll always keep feeling so lousy?

I’d like to report that I’ve found a way out of the oscillations of my personal sine curve. But it’s an emotional equation I can’t solve. I keep wrestling with this paradox: I am better, and yet I am not; I am being healed, but in a way it doesn’t seem to matter. It’s possible that sarcoidosis has broken me to the point that eradicating the disease won’t fix me.

Without a solution, what do I do? For now, I am falling back on the single most important lesson I’ve gleaned from my eleven years in Chronic Town: run away from big concepts like cures or permanent damage and reside instead in each moment. I’m trying not to give up on hope, but just scale it back. I’m seeking refuge in daily life— in my son and my husband. I cannot solve a conundrum. But I think I can live within it.

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The Land of In Between

November 18, 2014 at 10:24 pm (Uncategorized) (, , , , , , )

I got a haircut a few days ago. This means I am maintaining my super short, post-chemo look. Andrew calls it a “miniature pixie cut.” Most people who see me—and get over not recognizing me without the long, red hair that defined me for most of my life—tell me that they like my new style. I like it too. I think short hair is flattering on me. Plus, it’s certainly much easier to get ready in the morning now. I literally wash and go.
Truthfully, though, I’m keeping my miniature pixie cut for a different reason. I can’t stand the prospect of growing out my hair. I’m daunted by the prospect of months of “in between” hair that is unmanageable.

There’s a lot in my life that feels as though it is caught in the land of in between. Take my health. I’m certainly much better than I was a year ago, when I was hospitalized for over a month. I was in constant, crushing pain. My vision was blipping in and out, and I could not walk across the room to the bathroom because my vertigo was so bad. I’ve come a long way from those hopeless days. Thanks in large part to my new drug regimen of Acthar (generously provided to me by the folks at NORD, the National Organization for Rare Diseases), I’ve stayed out of the hospital and have mostly been able to live a limited normal life.

This is great news, I know. But it’s not always easy. There’s a lot of pain and uncertainty in the “mostly” and “limited” qualifiers I used to describe my healthier reality. I’m still not able to work with any kind of regularity. The sporadic postings on my blog are a testament to the problems I continue to have with chronic pain, exhaustion, and neurological problems. The past few weeks have been particularly hard. For no reason that I can discern, my systemic sarcoidosis flared up once again, and I was stuck in bed at home for days on end, unable to read and in so much pain that turning over in bed felt impossible.

There aren’t many role models for living through these in between phases of life and illness. I’ll use one presentation of illness as an example. On the TV drama Parenthood, Kristina gets cancer. As in most other pop culture representations of serious illness, Kristina’s travails follow a familiar arc. She is diagnosed with breast cancer, goes through chemotherapy, and loses her hair. For a few episodes, she is literally fighting for her life. But she pulls through. Between seasons, the show moves forward a year in time—at which point, Kristina’s hair has magically gone through the messy, unsightly process of growing from totally bald into a lovely bob. We don’t see her wondering whether to wear a hat when her hair has grown into a crew cut, or what to do with her miniature pixie cut.

The lack of nuance in the show’s representation of Kristina’s illness transcends hair styles. By the time the narrative picks up a year after she is declared cancer free, Kristina is totally back to normal. In fact, she seems healthier than before her cancer. Not only does she have the energy to continue as a full-time stay at home Mom, she decides to run for mayor. She suffers none of the physical after-effects of her illness. She’s not tired from her months of chemo. When she’s delivering her moving campaign speeches, she doesn’t struggle with aphasia or chemo brain. As in so many other depictions of illness, she is either sick or she is well. There is no in between.

Watching something like Parenthood’s dramatization of cancer can be particularly demoralizing for those of us with a chronic illness. We frequently dwell in the shadowy land of in between, caught between illness and health, or see-sawing between periods of wellness and sickness. It’s the chronicity of chronic illness that I find most challenging. When I emerge from weeks of a flare-up, I want to be purely well. I certainly don’t want to have to go in for yet another monthly chemotherapy infusion, or deal with feeling somewhat better. Hell, I want to run for mayor—or, at least have the energy to cook dinner a few nights a week and write every day.

Chronicity is hard, and it’s not the stuff of gripping narrative. “Yesterday I felt mediocre. Today I feel maybe a little more mediocre. And tomorrow I expect to feel more or less mediocre.” It doesn’t make for great television. But life is not television. I have no choice but to live with chronicity, to keep plodding along in the land of in between.

I am going to keep my hair super short for the foreseeable future. That means I’ll have one less aspect of my life up in the air and in a state of flux. Miniature pixie cut, it is.

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The Hunger Games

June 20, 2014 at 10:57 am (Uncategorized) (, , , , , , , )

I’ve lost 76 pounds. Only 24 more pounds to go, and I’ll be back to my weight when I was first diagnosed with sarcoidosis and started on high doses of prednisone to treat the disease in my heart. I never thought I could lose the hundred pounds—or a significant percentage of them—I gained in Chronic Town. Even doctors told me it was impossible. “Not on that dose of prednisone,” the White Coats said.

I once heard a doctor describe prednisone as “the devil’s wonder drug.” It truly has a host of wonderful applications. Children with severe asthma are alive today because of prednisone, or so I’ve been told. But prednisone never cured me of sarcoidosis. In fact, it didn’t even seem to curb the disease’s progress. As though mocking the up-to-80 mg. of prednisone a day I’ve had to take, the sarcoidosis hop-scotched from my heart to my liver to my brain to my bones to my skin. Still, the doctors kept me on the prednisone for fear of what the disease might do otherwise.

The devilish aspects of this so-called wonder drug are its long list of side effects, ranging from loss of bone density to a slowed metabolism to a compromised immune system. It can rearrange the body’s fat distribution, causing the “moon face” and “prednisone hump” that are iconic markers of the drug’s use. But the side effects everyone talks about are increased appetite and weight gain.

Prednisone causes an unholy hunger that is unlike anything I’d ever experienced before. I’d eat a full meal and an hour or two later I’d feel this gnawing inside me that demanded attention. So I’d eat a giant bowl of cereal, which felt like throwing a teaspoon of water on the volcano of appetite uncoiling within me. Prednisone hunger felt like it might kill me. So I ate. And I gained 100 pounds.

My weight-loss started last fall, when I was in the hospital for over a month because of a neurosarcoidosis flare-up. I was so sick and in such pain that I couldn’t eat for days. Anything I’d put in my mouth I’d vomit right back up. At the same time, the years of chemo and other medications finally caught up to me and caused me to develop an ulcer. By the time the doctor had gotten my pain under control and I was ready—and prednisone hungry—to start eating again, my body rebelled against food. Every bite caused burning and cramping. It physically hurt to eat. So I stopped. I’d lost 20 pounds by the time I left the hospital. And I’d learned that I could live with the screaming hunger inside of me. I lost 56 more pounds without the aid of an ulcer to make eating physically revolting.

When people see the newer, slimmer me, they ask, “Did you finally get off prednisone?” No. I’m still on the ridiculously high “maintenance dose” the White Coats deem necessary. So how did I lose all this weight? The short answer is that I learned to live with the hunger. I discovered that it wouldn’t kill me. I also found that I was eating to try and fill a void that no amount of food could ever sate.

It’s true that I was eating because I was physically hungry. But I was also eating because I was emotionally hungry. It felt like sarcoidosis took away nearly every aspect of my life. For months, when the neurosarcoidosis was at its worst, I became an invalid, unable to get out of bed. I could not work, or be the kind of parent and wife I wanted to be. The disease also stripped me of the casual physical pleasures that had once sustained me. Stuck in bed, I could no longer go for an evening walk or a brisk hike. The gym felt so inaccessible it might as well have been in Kathmandu. I also hungered for social interactions. Being so trapped at home meant that having lunch with a friend was a rarity—and, with my diminished energy levels, the day’s only accomplishment. Food was the last thing left I could indulge in, the only tool left to fill the ravening hunger for life within me.

Not much in my life has changed. Sarcoidosis still leaves me craving the pleasures of the life I once lived. I’m still on prednisone. Yet stuffing my face with cereal won’t make it better. Succumbing to the gnawing of my body and mind won’t help.

Sometimes you just have to live with hunger.

Have you ever struggled with a physical or emotional hunger?

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Chemo and Sarcoidosis Brain—Or Is It Brane?

May 30, 2014 at 4:33 pm (Uncategorized) (, , , , , , , , )

When I was writing my last blog entry I got stuck on the verb “does.” As I went to type it, my mind seemed to empty itself of my past fifteen years as a professional writer, along with nearly forty years of reading books and writing papers all stuffed with the word “does.” I paused. Does. How the heck do you spell does? The pause lengthened to an uncomfortable minute or two, in which my mind grew even blanker. I typed “dos.” Was that right? It didn’t look like it. So I tried “dose.” “But that’s dose!” I thought indignantly.

By now the blankness had given way to full-fledged panic. “I can’t spell does! What is wrong with me? Am I losing my mind?” With tears in my eyes, I googled “dos,” the closest I could come to the elusive “does” and came up with a lot of pages about disc operating systems and the number two in Spanish. Through my frustration and fear, I thought to try “definition do.” At least I remembered that “does” is one of the conjugations of the verb “to do.” And there it was. Third person singular. D-o-e-s. I slapped my forehead and groaned. Of course. How could I have forgotten something so basic?

The answer to my question is chemo brain, which the Mayo Clinic helpfully defines as “a common term used by cancer survivors to describe thinking and memory problems that can occur after cancer treatment.” I’m not a cancer survivor or a cancer patient. I’m a resident of Chronic Town, living with sarcoidosis for the past decade. But I’ve sure been getting a lot of cancer drugs in the past few years. Since 2008, I’ve been on a cocktail of treatments that includes at least two chemotherapy agents. My brain is finally bearing witness to the rigors of this protocol.

I’ve been having memory problems for a few years. I simply cannot remember names when I’m introduced to new people. Minutes after shaking someone’s hand in introduction, her name evades me. Similarly, I can’t keep track of appointments anymore. I used to keep my calendar in my head. These days, I’m lucky if I can somehow remember to look at my calendar to see what I managed to write down in it. Even leaving it smack in the middle of the kitchen table for me to see first thing in the morning doesn’t always do the trick. I’ve had to put Jay in charge of keeping track of my doctor appointments. I don’t lose my car keys, but that’s because I literally run into the counter where we stow our keys when I walk into the house. But I do lose whole conversations with Jay, Andrew, and my friends. I’ve taken to schlepping a notebook around with my everywhere to write down details of chats as they happen, but the odds are good that when the moment arises, I’ll forget I have my notebook.

But lately, my memory and thinking problems have reached a whole new level. I can’t remember words. And one day, as I was poised to write a check, I could not conjure up what year it was. I stood there shuffling my feet. Was it 2012? That didn’t sound right, but neither did 2013 or 2014? There was no way it was already 2014, right? After a few agonizing minutes of this, I somehow thought to look at my cell phone, which handily displays the date. It was 2014. Wow.

I’ve talked to my doctors about these memory chasms (lapses feels too small a word to convey the level of terror they bring with them). It’s all perfectly normal, the white coats reassure me. But that doesn’t make me feel better when I’m unable to place myself correctly in time and remember words I learned how to spell in first grade. Andrew tried to console me when I told Jay and him about my “does” debacle. “It’s OK Mom. The other day I spelled “know” as “now,” he said sweetly. But it’s different. It’s so different. Words are my identity. I belong to a tribe of wordsmiths, and to suddenly lose the most basic of our basic building blocks feels threatening and horrifying. Who will I be if I can no longer summon the words to write?

My doctors say most of my cognitive issues will resolve once I’ve been off chemo for a while. When will this be? Unlike most cancer patients who follow a protocol, I’ve been put on three chemotherapy drugs indefinitely. Hopefully, the Acthar I just started (thanks to the generous folks at NORD) will allow me to get a break from at least one of these toxic treatments—but that’s assuming the Acthar works (which we’ll hopefully get at least a preliminary sense of in a couple months).

So what can I do? I’m trying humor. I told the woeful tale of “does” as a joke to Jay and Andrew. How else can I present it? We deal with enough crappy side effects from my disease that manufacturing a crisis about how to spell “does” doesn’t feel fair to any of us. I also tell myself that every one of us alive is in Chronic Town. As we get older, our memories fade a little. It’s normal. It’s natural. I’m not being singled out for persecution. And there’s not much to do about it but deal with it, right?

I’m contemplating a chain of post-it notes, reminding me to look somewhere else to be reminded of something important. I can stick post-it notes on the bathroom mirror, the coffee pot, and my forehead to prod me to look in my calendar so I can remember that I have yoga on Thursday evening and a coffee date on Friday. If more basic words continue to fail, I’ll start a file of them on my computer, along with the year. Now, if I could just remember that I have the file, I’ll be in business.

Have you ever forgotten anything terrifyingly basic? What are your strategies for dealing with forgetting?

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Miraculous

May 20, 2014 at 9:01 pm (Uncategorized) (, , , , , , , , , , )

It feels like a miracle has happened to me.

When I last wrote, I was stuck between good and bad news. The sarcoidosis guru I see in Ohio recommended a promising new treatment for me. But the plan managers for my prescription drug plan refused to cover the expensive medication called Acthar. Since then, we went through two rounds of appeals with the plan managers, and got a resounding “NO” for our efforts. In fact, the bureaucrats wouldn’t even talk to my doctor. A “licensed pharmacist” made the decision that I should try IV prednisone—as if I haven’t endured numerous rounds of that over the past decade.

We had one round of appeals left. Unfortunately, the plan managers told us that even if we somehow convinced them to approve Acthar, they could opt to classify it in their pricing scheme so that we would have to pay for half the medication – which wouldn’t even count toward our annual out-of-pocket maximum. At somewhere around $30,000 a vial, such a victory over the plan managers would be a pyrrhic one. There is no way we could afford one month of such “coverage.”

I think I stopped taking real breaths after Jay got off the phone with the plan folks and told me the news. For the first time in many years, my doctor had a treatment that he thought might actually help me. After 3 months of Acthar, I could at least (hopefully) get a break from Cytoxan, the chemotherapy infusion I get every month. It felt like just as I heard the first good news in quite a while, faceless and nameless bureaucrats were snatching it away.

Here’s where the miracle comes in. The sarcoidosis guru put me in touch with a non-profit called NORD– National Organization for Rare Diseases (https://www.rarediseases.org/). In addition to providing advocacy and education for those affected with rare diseases, including sarcoidosis, NORD offers patient assistance programs. One such program helps patients obtain so-called “orphan drugs”—medications that treat rare diseases and so are not manufactured in large quantities and are often extremely expensive—like Acthar. I filled out an application, crossed my fingers, and kept holding my breath.

I found out yesterday that NORD approved me for full assistance so that I can obtain a 9-months’ supply of Acthar at no cost. I am overwhelmed with gratitude to NORD and its donors who made this possible. I can’t put into words how grateful and we relieved we are. I finally took a real breath. We can postpone our battle royale with the plan manager. I can try out this new treatment, and maybe get a break from the awful side effects of the Cytoxan. It brings tears to my eyes every time I think about it. All I can say and feel is “thank you, thank you, thank you.”

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