A funny thing happened this summer that has altered the course of my life from here on out.
It began in the middle of the night, attacking like an intruder in the dark while I was asleep and unable to discern reality from my dreams. A stabbing pain in my right ear brought me upright, but movement made it worse and my hand flew to my ear instinctively to protect it as I dropped back to the pillow in confusion. I held perfectly still, staring at the wall in terror, unable to turn my head or move my jaw to even call out for help to wake my husband. Fear welcomed the worst scenarios into my imagination- was this a stroke or an aneurysm? Was I dying?
The pain was not entirely unfamiliar. It was inside my ear, much like the searing throb of a severe ear infection, but larger somehow. When I touched the skin over the opening of the ear canal, the pain was instant and I retracted my hand as if scalded. I recalled sleeping on my ear wrong in the past and feeling something similar, though not as painful, and it was short-lived. I assumed this was the same thing, and was reassured when I woke in the morning to find the symptoms were gone. It occurred again the next night, lasting for a few hours, and again the night after. I let this continue for two weeks until the morning it did not go away and I suspected it must be an ear infection. I took ibuprofen and promised myself I’d stop by urgent care after work, but that evening the wait was four hours or longer. The helpful receptionist suggested an e-visit instead, and I filled out the online form to wait for a robot (I assume) doctor to respond while I remained in the waiting area. It did not take long, but the instructions were underwhelming. Take ibuprofen and see a doctor if symptoms persisted. I couldn’t wait four hours; my morning alarm would go off around 4am, so late nights are impossible. I went home instead and spent another sleepless night wishing I had just waited.
Morning again brought little relief and I felt dazed and foggy as I dragged myself to the car on little sleep and in agony. I was halfway to work when I realized I was having a focal seizure; I was aware and fully able to continue driving, but everything was eerily familiar down to the cars next to me and the thoughts and emotions in my head, and I was sure I had just dreamed it all recently. This passed in a few minutes, but it had been many months since my last focal seizure and the occurrence scared me. Not ten minutes later, I was struck with sudden vertigo and I took the exit for the hospital.
The emergency room doctor spent a few seconds checking my ears before announcing it was an infection in both the middle and outer right ear. She prescribed antibiotics and antibiotic drops. We both thought it odd that I had no sinus symptoms that might cause this, but a month earlier I had been swimming, however briefly, in the lake at my mother’s home. I couldn’t remember putting my head in the water, but maybe one of the kids had splashed me? We had our scapegoat for swimmer’s ear and were both satisfied.
The antibiotics helped immediately and I was content for about a week, but a day after finishing the last tablet, the pain was back with a vengeance. This was nuts! I thought. I made it through a pandemic without so much as a cold and now I can’t kick a common childhood ailment? I tried another e-visit, thinking I just needed more antibiotics, but again the robot doctor online refused to diagnose me and directed me to an urgent care near work instead.
I have a long list of unrelated aches and pains that have earned me the award for biggest hypochondriac in my extended family. The most recent among these had led me to demand a referral to a rheumatologist to uncover the cause of body-wide swelling, multiple aching joints, a random case of scleritis in one eye, and excruciating pain in my right shoulder blade that just wouldn’t leave after months of patient tolerance. Dr. Snell was unimpressed with the minimal bloodwork and x-rays she ordered, but I had a standing order to check labs for inflammation the next time I really began to hurt. With everything going on this morning, I decided to get the lab work before swinging by urgent care.
The triage doctor was not very excited when he checked my ears this time and assured me I did not have an ear infection. I began an internet search of my symptoms while sitting in the waiting area for the next hour. My husband and I had a long weekend getaway planned in Vegas in a couple of days- our first real vacation since the pandemic had started- and my biggest concern was making sure I didn’t need to find antibiotics while I was out of town. When I was finally called back to be examined, the physician looked in each ear and immediately prescribed more drops for swimmer’s ear. She gave me three minutes of her time, scoffing at me with a raised eyebrow when I asked if there were anything else it could be, before showing me the door. I began to feel better after more ibuprofen and drops, and decided to head in to work.
Within hours, I received a brief email from my doctor following up on the results of my bloodwork.
“I suspect you may have a rare autoimmune disorder called Relapsing Polychondritis.”
I googled it and my stomach lurched. The ten year survival rate was estimated between 45% and 55%. The disease course can damage numerous organs, resulting in blindness, deafness, a disfigured saddle nose, disfigured floppy ears, tracheal collapse, and often death when the respiratory or cardiovascular system is affected. I reread the email to make sure I was typing the name correctly. Surely a reputable physician wouldn’t just throw something that devastating into a two line email and write a prescription for prednisone without even a phone call. It seemed so absurd!
I texted my husband and he pulled up the same statistics, but we both agreed the doctor must be crazy. I don’t have a saddle nose (I double checked my profile with my phone camera, obviously) and my ears weren’t red. I just had pain inside my ear at night, more like a severe ear infection that comes and goes. It didn’t even really hurt during the daytime. That could be anything, right?
I picked up the prescription on my lunch break and headed back to work in a daze. The moderate 20 mg dose of prednisone kicked in quickly and I noticed within a couple of hours that the persistent swelling in my hands, a constant presence for months now, was reduced. By morning, all my joint pain was gone and I felt like a new person. Aches I previously attributed to growing older had disappeared, including two decades of progressively worsening back pain and a severe ache in my right shoulder that had grown unbearable in recent months. I spent a few minutes that day looking up symptoms in between work meetings and decided the rheumatologist was mistaken; out of pain and feeling the euphoric buzz of prednisone, I felt great and redirected my focus toward the first vacation we had taken since the pandemic hit 18 months earlier.
Four days in Las Vegas in August is not the ideal vacation for a woman who doesn’t gamble, enjoy the heat, or like shopping, but anywhere was better than continuing to spend every weekend at home. As I’m also partial to a good margarita next to an air-conditioned replica of a Venetian canal, I found the trip to be just what we needed to celebrate the end of an unvaccinated era.
Our last night there, I noticed the prednisone wasn’t working as well, and the next day I felt generally miserable as my joints hurt and my muscles ached. My doctor had prescribed a standard tapering dosage, starting at 20 mg of prednisone and reducing this by 5 mg every five days. This was the first day on only 15 mg. It wasn’t until the plane was landing in Portland, though, that I really began to worry. The pressure change as we began the descent caused immediate stabbing pain in my right ear. Once on the ground, both ears began throbbing, a new symptom; what began as an ache akin to an ear infection spread to the cartilage in the ear canal, stopping just short of the visible auricular cartilage. This was the first time the onset of pain occurred in the daytime while upright instead of at night.
The next morning, the pain had spread to the cartilage behind my ears and I was faced with the hard truth that my rheumatologist may be right. I wanted to remain in denial, but everything I read pointed me back to the same conclusion: I have Relapsing Polychondritis.
Three months later, I don’t yet know how this journey ends. Every new turn in the road usually begins that way, though. I don’t know if I’m ready for this one, but here we go.
Further reading if you’d like to learn more about Relapsing Polychondritis:
Relapsing Polychondritis: An Updated Review (nih.gov)
Relapsing Polychondritis – NORD (National Organization for Rare Disorders) (rarediseases.org)
