In Amber

A DECADE

A decade since I felt well.

A decade since my body and health were not on my mind. 

A decade since my last cold, flu or bronchitis.

A decade since my last vaccination.

A decade since I enjoyed Halloween, my favourite holiday.

A decade since I was in a lake or ocean.

A decade since I was on a train.

A decade since I stood up at a concert.

A decade since I didn’t wear a mask on a plane.

A decade since I went to a wedding.

A decade since I went to a barbecue.

A decade since seeing so many friends.

A decade since I married my longtime boyfriend because “I feel like something is going to happen to me and I want you to be able to legally speak for me, if I can’t speak for myself.”

A decade since I was in Ireland, in my childhood home, walking the streets of my heart.

I thought about this anniversary so many times in the past. For a long time, I thought there was no way it would come–I couldn’t possibly stay sick this long. Every other illness had a beginning and an end, so, surely, one day my body would recover and this spectre would leave, it was just taking a little longer than the usual virus.

Once I realised it was lifelong, I thought the 10-year mark would be a momentous and heavy occasion. It turns out, it’s not. 2 years seemed much harder to accept. Back when isolation was still harrowing and loneliness still suffocated. You get used to both. It helps if you can develop a deep disdain for humans, so you can trick yourself into believing you’re not missing out on anything. And the 5-year mark was hard. I’d felt small, but miraculous changes from IVIG and then had an epic autumn backslide that year. The dowsing of that little flame of hope was devastating and it was inconceivable that I would be physically or mentally resilient enough to continue the maybe-I’m-getting-better!-Oh-no-what-fresh-hell-is-this? cycle for years to come.

But, then, suddenly, 10 years have passed. I could almost believe the rest of the world is trapped in amber, frozen in time, awaiting my return. As soon as I kick this thing, I’ll drive back down to the office–each street scene melting and returning into motion as my car passes by–and get back to work. Thanks for waiting, guys.

INFECTIONS

What’s far more unbelievable to me is that I haven’t had a muggle illness in a decade. [Please don’t let this jinx me.] No head cold, no flu, no stomach bug, no chest/ear/sinus/bladder/any-other-part-of-the-body infection. The more time that went by, the more ominous was the thought of contracting an acute virus. For years, I had relentless flu-y symptoms–headaches, sore throats, muscle pain, weakness, chills (and still do, sporadically)–and I have many high out-of-range infection titers*, so the thought of another malady compounding the daily slog was harrowing.

*HHV6 IgG; HSV IgM; EBV IgG; M Pneumoniae IgG; S. Cerevisiae IgG; Varicella IgG and IgM; Coxsackie A7, A9, A16, A24, B1, B2, B5 and B6; Anti Streptolysin O Titer, and Candida IgM and IgA. Yes, really.

Three years into my illness, Dr. Chia told us unequivocally that a run-of-the-mill cold could make me permanently worse, so we have always taken great precautions to avoid exposure, which have only intensified during this pandemic. I honestly wonder if I’ll ever be indoors and maskless with anyone besides my husband again. Even worse, will my husband ever be indoors and maskless with anyone besides me? It’s one thing to choose this life for myself–I’ve made peace with only having remote communication with friends and family; I have a partner and a dog to keep me sane–but my healthy husband’s life has shriveled to keep me safe and the guilt from that is indescribable. I imagine if he weren’t yoked to someone at such risk for serious complications from viruses, he might be out gallivanting and socialising, as well he should be.

VACCINES

One of the first doctors I saw after falling ill said, “You are very sick. We don’t know what’s wrong with you, but you should never get another vaccination as long as you live.” I was confused because, until that moment, I hadn’t linked whatever this sickness was to the flu shot I’d gotten a week before Halloween. I was also confused because, in my healthy ignorance, I thought vaccines only bolstered your immune system. I really didn’t understand, in certain unique circumstances, that they could break it. I used to get every immunization available in an effort to protect myself.

Before I traveled to Central America, I was vaccinated for polio, live typhoid, hepatitis A and B, tetanus, diphtheria and gammastan–all on the same day. In the years after, I got the live varicella vaccine, the 3-shot hepatitis B series, measles, mumps, and rubella and, of course, the flu shot every year, along with a pandemic flu vaccine (H1N1) when they were offered. I didn’t get majorly sick while traveling, I didn’t get chicken pox when I tended to my horrifically poxy husband and I never got the flu despite working very long hours in restaurants, among infectious people (note to the public: restaurant staff work when they’re sick; you have to be on death’s door to ask someone to cover a shift), so I guess the vaccines helped… until they harmed.

COVID

There’s such polarization these days when it comes to covid vaccines. There’s a lot of hatred directed at those who are trying to protect themselves and others by getting vaccinated and wearing masks and there is an equal amount of contempt directed at anti-vaxers. I have yet to see a single news story talk about those of us who want to get vaccinated, but cannot. Or those of us who have to make the agonizing decision to live a life of extreme isolation or risk very serious repercussions from a vaccine–any vaccine. I wish individuals would always take the collective into consideration and try to protect those that are vulnerable, but that’s not human nature, unfortunately. People will refuse vaccinations or not wear masks or not get tested because they don’t want to quarantine. And, all the while, those of us with weakened, damaged or overactive immune systems–be it from chemo or old age or autoimmunity or ME/cfs or steroids or stress or another condition–will have to choose seclusion over risk.

Against one of my doctor’s advice, I will be getting the first covid vaccine next month–but I’m getting a pediatric dose. We will see how I do and then I’ll get a second dose and test antibodies. They will be keeping me for observation in the clinic for an hour because of my history of anaphylaxis, but that’s not what I’m worried about.

I’m worried about being bedbound again. I’m worried about volunteering for an injection that could further damage my autonomic nervous system and intensify dysautonomia and hyperadrenergic symptoms. I’m worried about triggering more vasovagal collapses or making myself more hemodynamically unstable. I’m worried about a cytokine storm and/or a mast cell meltdown that creates a permanent worsening of reactivity when I’m already so limited in medication options and trying so hard to keep on weight. I’m worried about a blood clot causing sudden death because IVIG, oral hormones and inactivity already put me in a high-risk category. I’m worried about losing the limited amount of independence and mobility I have now (but it’s enough for a happy life) and becoming too weak to even wash my hair again. I’m worried about being that burden to my husband again, especially now that it takes so much work to make my GI tract function–it would be a monstrous task for him to take on. Mostly, I’m worried about once again losing the small joys, like taking Penny on our scooter walks, being able to talk on the phone for hours and laughing. I spent years without those gifts and I’m not sure I can claw my way back over another decade. 

It feels good to write out those fears. There are very few people with whom I can have these discussions because not many healthy friends understand the risks involved when dealing with such complex conditions. Everyone in my family has had at least 2 covid vaccinations with no side effects, but, in my support groups, it’s a different story. Even there, though, I am careful–I want everyone to get vaccinated, if they can safely, and I never want to dissuade others by voicing my concerns. And doctors aren’t much help because the vast majority take the practical stance that, statistically, the chances of negative repercussions are low and that the risks outweigh the benefits. That’s absolutely true for the typical bell curve of the typical population. Not true for me.

Let’s just hope it’s all smooth sailing. I’d like the next decade to be different.

Update: My doctor pretty much talked me out of getting the C vaccine. I’m too high-risk for long-term reactions. I’m going to have to get surgery next year, so not being vaccinated in a hospital setting adds another layer of fear, but I won’t be stable enough for surgery if the vaccine caused damage. So I will be remaining in strict isolation and putting my life in other people’s hands.

Title Credit

Halloween Update Litany

I’m going to try to write something. An update of sorts. Not necessarily because today is exactly nine years since this illness stepped into my body and started controlling the trajectory of my life, but more because it is a quiet Saturday and I can’t call any clinics and I don’t have any medical appointments. It’s Halloween, but we’re completely ignoring it this year. It’s a beautiful day, but I woke up after five hours sleep with bad brain symptoms, so I’m not up for going outside or calling a family member or washing my bed clothes, which are in dire need. And I’m just so tapped out on research right now. Endless, endless research into treatments and specialists and ways to bankrupt ourselves on nifty devices that might miraculously give a reprieve from symptoms or plateau my decline in functioning.

I often don’t write — even if I have the time and energy — because I feel like I want to express something meaningful and express it beautifully, or at least express it well. Express it in a way that others might identify with it or even be moved by it. Or, if not meaningful or moving, I’d like to be able to write something informative. But that takes more mental energy and creativity. I always find a reason not to tap into the emotions that are necessary to write deeply and thoughtfully. I stay sane with distraction, coasting along a wave of TV shows and dog cuddles, trying not to look into the depths below. I’m finding distraction harder this year.

After five years of a slow, but fairly steady increase in functioning, I’ve gone downhill. Not because my dog died or because wildfire smoke was choking us for weeks or because I can’t see my family and my one friend who kept me sane by visiting regularly. And not because of the emotional toll of the pandemic and the rage and heartbreak caused by the political strife in the world. That’s all just icing on the distress cake. The actual bulk of my cake is made of pain, exhaustion, reactions, and failing organs and bones, with thin, bitter layers of isolation and future worries between the tiers of sponge. It’s a really unpalatable cake.

When I first met my friend Jak over at Mast Cells & Collagen Behaving Badly, she had been through ME, then she was dealing with mast cell disease and her body had started to have problems from EDS (Ehlers-Danlos Syndrome, a connective tissue disorder). Most people I’ve met with this illness trifecta started having mast cell reactions after ME hit them. I was the other way around. I’d been dealing with angioedema since I was teenager and I first went into anaphylaxis in 2001. ME hit a decade later. When I met Jak, I didn’t have an EDS diagnosis and, when I first got it, I ignored it and decided it wasn’t true. I remembered, though, that Jak had said, “I could have told you that. With some of your symptoms, it seems obvious.” Her pain and subluxations didn’t start in earnest until she was in her 40s and dealing with peri-menapause, so she cautioned me that EDS could raise its head in the future. No, no, I’m not hypermobile, I said and I ignored it. Well, there’s no ignoring it now.

Last year, I went back to the top EDS doctor here in Seattle and told him I hadn’t believed his diagnosis and could we start from scratch, work me up again, see if he truly thought I had EDS? He smiled (good doctor), he agreed (did another physical exam), he reiterated that I had EDS and showed me what my body is not meant to do. He also diagnosed thoracic outlet syndrome (TOS). For all my research, there are so many odd things about my body that I barely notice. It’s like whack-a-mole — I’m just trying to push down whatever the most concerning thing is on any given day. What do I care if I can’t hang my clothes up or hold my phone to my face without losing circulation through my arms and having my hands go numb? It’s really not important in the grand scheme of this illness. You adapt. So when the doctor asked me if I felt anything as he manipulated my arm, I said, “Nothing. Except there’s no blood flowing and I have pins and needles.” He smiled gently again. “That’s not nothing. That’s what I’m looking for.” It took a very long time for blood to come back into my forearm and hand after whatever he did and I had electric zaps for hours. That was a year ago and I still haven’t read about TOS or whether those symptoms are normal or what to do about them. I don’t really care right now because I’m too busy trying to whack bigger, louder moles.

My leg pain has gotten much worse. I can’t stand in the kitchen to cook as long as I could before and, anytime I do, I have to wear compression stockings and a back brace, but still need to go to the couch and lie down with my feet up after a short while, groaning with the effort. My neck and back have gotten worse. Something in my thoracic spine keeps going out and my lower back and tailbone have a constant steady ache. My neck always feels unstable, so I move it very gingerly, but it also always feels like rebar, so I try not to immobilize it. I pull a different shoulder or neck muscle seemingly every day, usually from thrashing around in bed (unfortunately, not in a fun way, not when I’m conscious). I’m currently ignoring a jaw ache and maybe a cracked tooth from clamping in my sleep and the fact that my eyes never stop burning and are sore when I move them. My left patella keeps shifting out of place and it’s agony when it happens, so I’ve been doing a deep-dive into knee braces and kinesiology taping. I broke my wrist and finger this summer when Penny lunged at an off-leash dog and snapped my hand behind my mobility scooter seat. I’m still wearing a cast or a brace a full three months later and my right hand, which picked up the slack when I couldn’t use my left, has developed instability in the wrist and a subluxing thumb. When my endocrinologist heard about my fractures, she said we needed an appointment asap because she is very concerned that my osteoporosis has progressed. She’ll probably suggest I take more drugs. 🙄

And really none of this is that important because it all pales in comparison to my bowel hell. Bowhell.

Warning: lots of talk about poop and toilets ahead. Enemas barely work anymore. To have a bowel movement, I have to use a liter of water and massage my abdomen for sometimes hours each day. And “massage” sounds delicate. It’s not. I often worry I’m going to rupture something with my squeezing. With my broken wrist and finger, I couldn’t manage to press my abdomen properly for weeks and my bowels suffered. I couldn’t evacuate effectively, which meant I couldn’t eat enough and I didn’t sleep properly. Everything has a cascade effect.

Compounding my bathroom issues is how difficult it is to sit on a toilet. I lose circulation in my legs very quickly (even with a Squatty Potty) and it is incredibly painful on my neck and back. My doctor asked me when my neck was the most painful and I realised it is sitting on the toilet because I have no support for my spine and nothing to lean back on. I’ve resorted to using a hard neck brace (only sometimes — sometimes it makes it worse) and putting a chair in front of me to lean my forehead against, but, even so, after I’m done, I have to lie flat on the floor and, if I have the energy, use heat, traction and ice to help the spinal pain. I was never conscious of just how much I need to support my neck until two years ago when my mother was visiting and I’d made enough improvements energy-wise to go to the opera. Wow, three hours sitting in a short-backed chair was excruciating. I was almost in tears. I was dizzy, my heart rate was high, my legs were losing circulation (I’m short, so I was using my backpack as a foot stool) and I could not hold my head up.

So, sitting is an ordeal. And shitting is an ordeal.

I have a long history of vasovagal collapse from abdominal pain. In my twenties, it happened with the onset of my period because of severe dysmenorrhea. Shockingly, when I got sick, my cramps virtually disappeared. But they’re baaacck! And my period often likes to come three times in one month, so this cramping and inflammation, coupled with random pelvic floor spasms, coupled with colon pain has been a lot. Last June, I sat up in bed one morning and some deep part of my lower abdomen spasmed and I immediately went into a vasovagal episode. My heart rate went so low, that I was having trouble breathing. My blood pressure dropped, too, but the main problem was the bradycardia. I was shaking all over and trying not to black out, but after about 20 minutes, I had to call the paramedics. Before they even got here, the pain abruptly ended and, instantly, my heart rate came up and I could breathe again. (I told them not to come inside because of covid and I gave myself IV fluids at home. I’ve dealt with this before, emergency rooms really can’t help.)

From that day forward, every day for six weeks, I was in an acute bowel pain crisis. I couldn’t seem to eat anything that didn’t contribute to the pain across my transverse colon, I lost weight, I wept each evening, I slept poorly, my attention was never not on this organ that was constantly yelling at me that something was wrong. I wound up getting a CT scan (a big deal during covid and when I’ve had so much radiation in my life) and blood tests because I thought: what if this is life-threatening? I was spooked by a fellow EDSer’s emergency surgery for a ruptured bowel and resulting colostomy bag, but I was even more concerned about the possibility of an elemental liquid diet or a feeding tube. I’ve gone to great lengths to keep a varied diet, not only because food is my one joy besides dogs, but also because I know so many people who never got foods back after strict and prolonged eliminations. And feeding tubes — I never want tubes of any sort stuck in my body, too many complications. It’s the reason I’m still doing weekly peripheral IVs after five years, rather than getting a port or PICC (I don’t know anyone else who has come close to tapping veins for this length of time).

The CT scan showed nothing except my big lunch and tampon (a mortifying radiology report: unremarkable, TAMPON, unremarkable, unremarkable, COPIOUS AMOUNT OF INGESTED MATERIAL IN STOMACH, unremarkable etc…) and the acute bowel pain eventually faded back to my regular constant ache with periodic stabbing knives and electric zaps. But it sure got my attention.

I started Motegrity, a selective serotonin type 4 (5-HT4) receptor agonist, which cost $265 for one box (bought online from Canada because my insurance balked) and then caused possibly the worst medication reaction I’ve ever had. I started Linzess, which cost $350 for one bottle and either causes nothing to happen or a full day of sharts. I’m still taking Iberogast, Miralax, BPC-157, SBI Protect, Thorne SF722, oregano oil, berberine, magnesium, digestive enzymes, betaine HCl, and probiotics… all for my bowels. I’m about to try Mestinon, LDN and Cromolyn again (okay, I take it back, the latter was actually the worst medication reaction I’ve ever had — and I’m going to try it again, which has to show my level of desperation); these are all medications that can help motility. Plus, I have a Xifaxan prescription at the ready (which I’ve already taken twice) when I’ve exhausted all of these options.

It’s a next level problem. What I mean is, there were four years in the beginning of this chronic illness when I was “just” dealing with ME and MCAS — when I could still poop! When it was “just” muscle pain, but my joints were fine and my bones felt sturdy. Unbelievably, there were years when I didn’t have brain symptoms. I had the low-level kind of brain fog that made you forget things or not be able to find words, but, in the beginning, I didn’t have the buzzing brain and eye pressure, slurring and screaming tinnitus that makes bed the only possibility, even if my body is feeling strong. These new additions take illness management to the next level.

I’m on my third gastrointestinal doctor. The first said: Miralax, papaya, probiotics. Huh? Did you even hear the part about dead colon? On a return visit, she said: Daily enemas for life. Are you fucking joking? I asked her when she would recommend a colonoscopy (back before I realised it would need anesthesia in my case). When you have bloody diarrhea, she said. Right. Okay.

The second GI doctor said: Colonoscopy and endoscopy. On a return visit, she said: COLONOSCOPY AND ENDOSCOPY. She would not talk about any other tests or interventions. I don’t want to go through that. I don’t think those procedures will show anything and, with my medication reactions, there are legitimate risks to full anesthesia, not to mention the clean out having risks because of my hypotension and hypoglycemia. I thought (and still think) that it was prudent to exhaust less invasive options first.

These two doctors were young women at the University of Washington, one touted as The Motility Expert and the other as being EDS-knowledgeable. I mention this because I would assume I would be most comfortable or have the best experience with them instead of the third GI doctor, who is an old man that made a slightly misogynist comment right out of the gates and doesn’t make much eye contact. But he has been the only one to think outside the box and marginally help me. He ran tests that nobody has ever run since I’ve been sick (I’d never had a stool sample done or celiac test!) and spent 40 minutes discussing my mast cell history before even broaching the subject of my bowels. He dismissed a colonoscopy and it felt like he’d lifted a 100-pound weight from my shoulders because I was crippled with guilt after rescheduling the procedures over and over for a year and a half.

So, because of the progression of bowhell symptoms and structural issues the last few years, I have been pursuing MRIs and neurosurgical consultations. A cine CSF (cerebral spinal fluid) flow study showed a lack of CSF in my hind brain, caused by low-lying cerebellar tonsils (LLCT). My neurosurgeon (who is experienced in dealing with EDS/MCAS/ME patients) also suspected craniocervical instability (CCI), but couldn’t recommend surgery from my MRI measurements and symptoms without first performing more tests (invasive cervical traction (ICT), where they lift up your skull with a pulley system to see if there is an improvement in symptoms, and intracranial pressure monitoring (ICP ), which is a bolt in the skull that holds a probe that measures pressure in your head while concurrently preforming a lumbar puncture). I decided not to do either of those because, as you might have guessed, I don’t like rocking the boat (with, say, a new soap, let alone invasive tests that involve holes in my skull) (oh, and travel across the country) (and covid). But I have wondered if the blocked CSF flow is contributing to or entirely causing my brain symptoms. That’s a big deal. I used to feel smart and effective.

But the biggest deal of all came from the neurosurgeon looking at my pelvic MRI defecography from five years ago (which I didn’t even send to him because I was only consulting him about my neck; he must have gotten it from my specialist, who was the referring physician).

“You have a large bowel,” he said.

“I’m not surprised.”

“And what have you been told about your enormous bladder?”

“My what?” He has a thick Italian accent straight out of central casting and I didn’t know there was anything abnormal about my bladder.

“Your enormous bladder. Your ENORMOUS BLADDER!”

He had to repeat it four times before I could understand what he was saying. It was pretty comical. Nobody had ever mentioned my bladder. He recommended a renal ultrasound to rule out hydronephrosis, urodynamic testing for neurogenic bladder, and a lumbar MRI to look for tethered cord. I’m sure you’ll be shocked to know, I ignored it all… until I watched online presentations by Petra Klinge, probably the top tethered cord specialist in the country, and a Q&A with Dr. Klinge and Jeffrey Greenfield where they mentioned that, although bladder symptoms are the hallmark of pediatric tethered cord, in adults it’s often bowel problems, usually constipation. Ah.

I sent my lumbar MRI (both prone and supine) to my neurosurgeon and he diagnosed “tethered cord, classic variant,” which is notoriously hard to see. In other words, as my complex disease specialist emphasized to me repeatedly, it is rare for this neurosurgeon to diagnose tethered cord before CCI from a lumbar MRI. This is the case even though they have both actively been trying to identify it early since so many of their patients have to return for a second “detethering” surgery after undergoing craniocervical fusion.

“Elizabeth, why aren’t you on a plane to New York for SFT [sectioning of the filum terminale]?” my specialist asked me.

Friends, I DON’T WANT SURGERY. EVER. No surgery, but ESPECIALLY NOT SPINAL SURGERY.

I will leave you there. We have much to discuss. This is now my focus. I need to do everything I can to manage these symptoms and to halt their progression. Meds, exercises, physical therapy, prolotherapy, I don’t know what. Right now, my plan is to plan. I’m not willing to see any healthcare practitioners in person, so it’s tricky, but it’ll be a winter of research and putting some ducks in a row.

I also have to start preparing for a what might be an inevitable surgical eventuality. I need a pain management protocol with bigger guns than paracetamol, I need to strengthen my core and my bones, I need to find muscle relaxants to which I don’t react, I need to get my blood pressure up and control my MCAS as much as possible. I need to save money. No more ignoring.

Happy Samhain, everyone. And my 9th “sickiversary” — not a happy day, but one that should be acknowledged, nonetheless.