When Horses are Zebras - A runner's struggle for diagnosis & treatment of a rare spinal fluid leak

- Stacy Kuebelbeck Paulsen

I remember the instant my spinal cord was ripped open.

It was January 15, 2018, one of those blindingly bright winter days so cold that the snow crunched beneath my car tires and squeaked under my shoes as I walked into the small strip-mall chiropractic office. After the obligatory paperwork, I found myself lying face down on the chiropractor’s adjustment table, my first visit with a new practitioner for lower back pain from a workout strain several months earlier. Opening a small gap in the table under my chest, the chiropractor placed her hands in the center of my back and asked me to take a deep breath and fully exhale. At the bottom of the exhale she pressed down, hard and fast, in the center of my back so that the air still remaining in my lungs came out in an involuntary huff. Surprised at the force used and embarrassed at the sound I had just made, I gave a nervous laugh and wondered aloud, “Is that supposed to happen?”

The chiropractor assured me that it was and proceeded with the rest of the adjustment, pressing here, tugging there, in an effort to alleviate the muscle strain that had been bothering me for months. I left the office a half hour later and rushed into the rest of my day, not realizing that my life was about to drastically change. The adjustment had torn an opening in the protective covering of my spinal cord at T10-11, roughly at the bra strap level, in my thoracic spine. As I made my way to work that day to greet classrooms full of energetic students, the fluid surrounding and cushioning my brain and spinal cord began to spill out of the opening, each precious drop causing my brain to sink lower in my skull. By that evening, I had a severe headache unlike any other I had ever experienced. Excruciating when I stood upright, it relented within a few minutes of lying flat. Only later did I realize why. The cushioning fluid that normally surrounded my brain, allowing it to float buoyantly in my skull, was leaking into my chest cavity.

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Growing up in a family of eight in rural central Minnesota, I had to seek out creative ways to be alone. In elementary school, I planned solo Saturday morning excursions into the woods and pastures surrounding our family’s home, packing a breakfast and getting up early so I could be outside before anyone else was awake. It was magical to see things that no one else was seeing because they were still asleep in their beds. There was never a possibility of waking before my father, as he was up and out of the house before 6 a.m. six days a week delivering milk to area homes and businesses, but he cultivated my appreciation of quiet and stillness on the Saturday mornings when it was my turn to help him on the milk route. We’d leave the driveway in darkness and silence, conversation only gradually picking up to match the growing light, absorbing stunning sunrises and glimpses of the occasional deer or fox on the drive to the first neighborhood on his route. At home, I spent countless solitary hours sitting in our tree house, writing in my journal and listening to the sounds of the wind or raindrops striking the leaves. I remember one wintry afternoon hike by myself, a mile or two into our neighbor’s woods through knee-deep snow, my legs so fatigued from lifting them above the snow pack that I was afraid I might not make it back to our house before dusk.

I craved the silence and contemplative time that these moments provided, and in my teens, running fulfilled that craving. I started running along the country roads near my home, relishing the views of rolling hills and fields of alfalfa and corn and the meditative rhythm of my footfalls. I was drawn to cross country in high school, but given the distance from school and lack of transportation after practices, joining the team wasn’t workable. So, I ran on my own. I ran when I was frustrated, when I was happy, when it rained and when it didn’t. Not fast, not far. But I ran. And I kept running through college, while studying abroad in Greece and Italy, around the Panathenaic Stadium in Athens, through the car exhaust and traffic near the Colosseum in Rome. I ran through my twenties, through graduate school, the morning of my wedding, through most of my three pregnancies and afterward, inside and outside, through workouts interrupted by hungry babies and squeezed in before dawn. I ran because nothing else could give me that euphoric feeling of health and vitality, because it let me blow off steam, reset my patience, put things in perspective, pray.  A million reasons. It didn’t always feel good while I was running, but it never failed to make me feel better afterward.

Luce Line Trail, Plymouth, MN

My husband, Bob, and I chose our house in Plymouth, Minn., a suburb of Minneapolis, partly because of its proximity to a beautiful running trail, and we logged hundreds of miles along the limestone surface of a former rail bed. Despite my passion for running, I didn’t run a race until my 40th birthday. When I finished that first 5K in 26:40, I wasn’t even sure if my time was impressive or pathetic. I went on to complete a few more races in my 40s, with my best 10K in 52:52. Not stellar, but respectable. I quickly realized, however, that racing wasn’t what running was about for me. It was about the solitude and the mental clarity, the feeling of strength and health. So I returned to my running routine without regard for races, churning out 3 to 5 miles every other day, interspersing running days with core and strength workouts I assembled over the years from yoga and Pilates classes and training spreads in running magazines.

Fast forward to fall 2017. Now a mom of three teenagers (runners all) and a newly minted science teacher after a career change in my 40s, I felt I had finally hit my stride. Our oldest daughter was in the process of applying to colleges; our middle daughter happily adjusting to high school and blossoming into a competitive cross-country runner; our son finishing up middle school. With a couple of years of teaching under my belt and my kids secure in their developing independence, I began to imagine the possibility of more time to focus on my own goals, to make time for writing and self-discovery, take some classes, try new things.

At age 49, I prided myself on maintaining a body that, aside from a few wrinkles and stretch marks, could compete with those of my teenagers in terms of strength and overall fitness. I could still ask my body to do almost anything I wanted it to do. Our family enjoyed running and hiking in the mountains together on our family vacations, paddle boarding on the lake down the street, and downhill skiing on Minnesota’s version of “mountains.”

Looking for a change of pace in between running days on a rainy September morning, I decided on a whim to try a workout video. I overdid it, and by the next morning the muscle strain was painfully evident. I tried the usual round of remedies: ibuprofen, stretching exercises and a course of steroids and muscle relaxants from my GP. No luck. The pain just kept hanging on. By January 2018, I was looking for relief and open to alternatives. Having had success with chiropractic in the past for some knee pain, I thought I’d give it a try again. Googling “chiropractors near me” brought me to that ill-fated January morning.

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"Frontal View" Matthew S. Cain CC BY-NC-SA 2.0 / Caption added

We are accustomed to thinking of ourselves as terrestrial creatures living a dry land existence, but our anatomy tells a different story. The most critical portion of our bodies, the part that processes stimuli, emotions, memory and cognition, and instructs our bodies to move is awash in fluid, perhaps a relic of our deep evolutionary past when life arose in ancient oceans. Our brains and spinal cords are entirely surrounded by cerebrospinal fluid (CSF), a salty, nutrient-rich broth. Continuously replenished by a structure called the choroid plexus deep within the brain, CSF normally circulates around both the brain and spinal cord, serving to buoy, cushion, nourish and cleanse delicate neural tissue. How delicate? A human brain has the consistency of Jell-O. Without the buoyancy of CSF, it would sink into the bottom of the skull, damaging itself with its own weight. CSF allows the brain to float in the skull, reducing the 1500g average adult brain weight (equivalent to one and a half liter bottles of soda) to a scant 25g, the weight of a AA battery. Similar to a football helmet, CSF also acts as a shock absorber to slow impact forces when there is a blow to the head.

With a tear in the dura, the leathery protective covering of the brain and spinal cord, this vital fluid seeps out. Like the drain in the bottom of a bathtub, the leak draws CSF away from the brain, especially with the added pull of gravity when standing upright. If enough CSF drains away, the brain can tear away from the inside of the skull, causing hemorrhage and seizure. The classic early symptom of a CSF leak is a severe positional headache, for some so intense that patients experience nausea, vomiting and fainting within seconds of standing upright. It is during this early, acute phase that patients have the best chance of a correct diagnosis. If untreated, the headache tends to moderate over time, with some patients experiencing constant daily headache regardless of position. Without the classic positional headaches, it can be nearly impossible to get practitioners to consider the possibility of CSF leak.

CSF leak sufferers also develop a constellation of neurological symptoms from “brain sag” and the resulting pressure on brain structures and cranial nerves. These include sensations of head pressure, hearing and vision changes, popping and ringing in the ears, numbness and tingling in the face and arms, pain between the shoulder blades, vertigo, heart rate and blood pressure changes, cognitive issues, and a laundry list of other symptoms. Faced with such a wide range of nebulous complaints and a headache that is no longer positional, it can be even harder for doctors to recognize that the patient’s symptoms fit with a CSF leak.

Medical school students are taught the old adage, “when you hear hoofbeats, think horses, not zebras.” In other words, the most common and obvious diagnosis is usually correct. A zebra, medically speaking, is a very “unlikely diagnostic possibility,” a diagnosis so rare that it hardly warrants consideration. CSF leaks are the zebras of headaches.

When patients present with an array of symptoms, CSF leak is usually the last thing doctors suspect. Accounting for only an estimated 0.5 to 1 percent of emergency room visits for headache, CSF leak patients are routinely misdiagnosed with tension headaches, chronic migraine, anxiety, chronic pain syndrome or my personal favorite, NPDH (New Persistent Daily Headache), the catch-all category for “we don't have a clue why this person is suddenly having headaches.” While the occurrence of “spontaneous” CSF leaks is estimated to be approximately five in 100,000 people in a given year, the frequency of misdiagnosis might mask the true scope of a much more widespread problem.

Barriers to diagnosis are even higher for women due to gender bias in diagnosis and treatment.  Women in pain are more likely to be dismissed as anxious or overstating their pain, less likely to be taken seriously, more likely to be prescribed sedatives and antidepressants as a Band-Aid for their pain, and less likely to receive the attention needed for correct diagnosis of the true source of their pain. These disparities are especially important for CSF leak patients who, for some as yet unknown reason, are most likely to be middle-aged women.

Adding to the nightmare of diagnosis for many patients, the rarity of this disorder means that most radiologists lack experience recognizing the telltale signs of a leak on MRIs and CT scans. Almost every leaker has had a handful of scans that came back “normal” simply because the radiologist reading the images was not familiar with the subtle diagnostic criteria for CSF leaks. Delays in treatment can stretch from months into years for some sufferers. The average time from onset to diagnosis is a debilitating 13 months.

The disability, lost work, and productivity for sufferers is incalculable. Symptoms prevent most patients from working, and many patients are bedridden. With only a handful of centers in the U.S. that specialize in treating spinal CSF leaks, there is the additional challenge of access and coverage, as these places are almost uniformly “out of network” for insured patients. Coverage is often a moot point because most patients need to get past their primary care gatekeepers with a diagnosis of CSF leak before they can even begin the process of engaging with CSF leak centers for care. The additional barrier of travel and out-of-pocket expenses required to be treated at these centers proves much too high for many, leading to extended periods of disability and suffering.

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"electrical hazard" by danna § curious tangles
is licensed under CC BY-NC-ND 2.0 / Caption added

At first, the grinding pain of the headache took precedence over all else. Anyone who has ever had a migraine knows that severe headache pain is uniquely incapacitating. Compartmentalizing the pain and going about my day was virtually impossible. A severe headache imprisons you inside your own skull.

After four days of agony, I went to see my general practice physician and was given the usual round of blood work, an X-ray to look for sinus issues, and an order for an MRI. While I waited for the results of diagnostics, I called on the mental and physical toughness gleaned from my running practice and tried to power through my usual responsibilities. A compulsive list maker, I relied on hyper-organization to manage the complexity of a five-person household. Thanks to my husband’s long work hours and nightmare commute, doctor, dentist, and orthodontist appointments, after-school carpools, meal prep, bill paying, housecleaning and most of the laundry chores fell to me. There were times I felt like a human sponge, absorbing all the tasks that no one else had time for, but it was an arrangement that mostly worked for us. With my headaches, all of that began to unravel. The human sponge had started to leak.

Prioritizing my job duties, my new “routine” meant lying flat as long as I could in the morning, grading and preparing lesson plans with my laptop before becoming upright, downing mega-doses of over-the-counter pain medications, and making it through my classes with a smile on my face through sheer force of will. I would get home and immediately go horizontal to dampen the continuous grinding ache that my entire head had become. Balanced, home-cooked meals were replaced by takeout and quick “dinners of desperation,” as I called them. Laundry stacked up, bathrooms stayed dirty, and things fell through the ever-widening cracks. I found that I could get some relief if I laid on my stomach with my head dangling down over the side of the couch. Looking back, this makes perfect sense, as it allowed CSF to pool in my brain and reduced the traction on the tiny connecting fibers attaching my brain to the inside of the skull.

A researcher by nature, I laid on the couch and started digging for answers. Slowly the pieces began to click into place. Having taught childbirth education at a local hospital for eight years, I was familiar with the possibility of headaches which can occur after women give birth with epidural anesthesia. Providing pain relief in labor, epidurals are administered with an injection of anesthesia in the space around the spinal cord. Occasionally, the puncture site causes a spinal fluid leak and severe positional headaches that can last for weeks. In a twist of irony, I had avoided epidurals for all of my deliveries, in part out of concern for these risks. Around this time, my mother shared the story of a neighbor who experienced debilitating headaches after spinal anesthesia for a surgical procedure. Although I had no history of epidural or spinal puncture, I began to suspect CSF leak as the cause of my headaches and started connecting the dots. My headache started just a few hours after that first high-velocity chiropractic adjustment. If I was leaking spinal fluid, the chiropractic adjustment seemed the likely cause.

"peridural anesthesia" by Stephan
Winkler is licensed under CC BY-NC 4.0 

I dug through the literature and found studies linking trauma of various kinds to CSF leaks: surgeries and lumbar puncture procedures, falls, car accidents — and, yes, chiropractic adjustments. Some studies pointed to the combination of trauma and bone spurs that develop on the spine as part of the natural aging process. These tiny, sharp spurs can rub against the dura and result in a puncture or tear, either “spontaneously” over time or abruptly in the case of some other spine trauma. Recognized since the early 1900s but only studied in recent decades, the etiology and symptoms of CSF leaks are still not fully understood.

A few stories of notable people popped up when searching for CSF leaks. Musician Austin Carlile, singer/actor Duncan James, and actor George Clooney all belong to the exclusive club of people who have suffered spinal fluid leaks. Clooney’s came after a stunt-related accident on the set of the movie Syriana in 2005. Clooney’s harrowing account of his injury and treatment leaves no doubt as to the gravity of the condition. He openly acknowledges thoughts of suicide during his ordeal to escape the pain and anxiety. In an interview, he described 2005 as the year in which he aged a decade. 

"George Clooney -Toronto Int. Film Festival 2011 1571" by
Marco Manna Photography is licensed under CC BY-NC-ND 2.0 

Armed with some background information about CSF leaks, I returned to the GP’s office in early February. In an effort to dampen the pain, I laid flat on the exam table before she even entered the room, my husband present for support. MRI results had come back “normal” and I was looking for next steps.

I mentioned my suspicions about a spinal fluid leak. She responded with a patronizing smile.
“We don’t see that clinically,” she said condescendingly, adding that there’s no reason to go searching for obscure explanations. “It’s quite common to develop sudden daily headaches out of the blue.” My husband and I exchanged glances and asked if we could get a referral to a neurologist. She agreed, and as soon as we reached our car in the parking lot we called to make the appointment. The neurologist’s next available opening was more than a month away.

I suffered through the next weeks, desperately trying to work and function. Running, my main outlet for positive mood and energy, was impossible. I tried a few treadmill runs, only to cut them short when I started to feel dizzy, my entire body vibrating like a tuning fork. Family obligations contracted to the bare minimum as I struggled to help our eldest daughter finish her college and scholarship applications. My upright hours shrank to only the time that I needed to be at school, on my feet, teaching. I would lie flat before work, medicate to teach, and barely make it home before collapsing back to horizontal.

During these weeks, other new symptoms began to appear. If I turned my head quickly, I could feel my brain ricocheting painfully against the inside of my skull. My hearing grew muffled, my ears felt pressurized and perpetually needed to pop and click, and a constant industrial whine rang in my ears. A gnawing ache developed between my shoulder blades, a stiff and painful neck made it hard to raise my head, and feelings of pressure and coolness developed in my chest cavity in the location where fluid was leaking. And, of course, the debilitating headache continued.

When I finally reached the neurologist, she ordered another MRI. The results yielded my first concrete evidence of a CSF leak. The outside covering of my brain that should have been invisible on MRI was lit up like a Christmas tree. This kind of “meningeal enhancement” occurs when tiny blood vessels in the brain try to compensate for the lack of buoyant surrounding fluid by becoming engorged with blood. It also explains why some CSF leakers experience brain hemorrhage, seizure, or stroke when these tiny vessels tear.  The rest of my symptoms fit the profile of intracranial hypotension, low cranial pressure resulting from a loss of CSF. The doctor advised conservative treatment consisting of partial bed rest, headache medication, and large doses of caffeine to stimulate CSF production. I dutifully embraced the regimen but continued to suffer.

In late March, while standing, hyper-caffeinated, in the Target checkout lane with a cart full of toilet paper and necessities, every system in my body started ringing alarm bells. My head was doing its continuous roar, my heart was galloping, I had chest pain, shortness of breath, and felt faint. I somehow managed to drive home and a neighbor drove me to the emergency room. My blood pressure and heart rate sky-high, I was transported by ambulance and admitted to Abbott Northwestern Hospital in Minneapolis.  After doctors ruled out heart attack and drug overdose, I was seen by a young neurologist who was, thankfully, at least familiar with CSF leaks. He ordered another series of MRIs, a myelogram to try to locate the leak, and an epidural blood-patch procedure.

Abbott Northwestern Hospital, Minneapolis, MN

In a standard myelogram, the patient lies face-down while a radiologist uses a large needle to inject contrast dye into the spinal canal in the lower back. Following the injection, the table is tilted to distribute the dye along the length of the spine. The increased pressure from the dye injection can exacerbate head pain and cause nerve discomfort — even seizure — if it travels too quickly to the brain. With my heart already racing at 140 beats a minute while I was lying still in the hospital bed, I had no interest in being awake and alert for this procedure. Unfortunately, I was told that patients have to be conscious in order to provide feedback about sensations, ensure proper needle placement, and avoid nerve damage. Nurses assured me that I could at least receive a mild sedative.

The next morning, I awoke to an orderly wheeling in a gurney, announcing, “I’m here to take you to radiology.”  When I inquired about the promised sedative, a nurse informed me that it was too late, offering instead a lavender-scented aromatherapy tube for me to inhale during the procedure.

“Lavender is actually highly effective for relaxation,” he assured me.

“So is morphine,” I thought, angrily. Pain, not relaxation, was my bigger concern.

Once in the radiology room, I was moved to the table and prepped for the dye injection, clutching the silly lavender tube in the hand curled near my face. The needle went in my back with a slight pinch and the ground shifted under me as the table was tipped. The pain that followed was immediate and excruciating. The pressure from the dye sent a knife of pain through one of my legs and seemed to suck all the sound out of the room as my hearing vanished. When the dye reached my head, I was paralyzed with pain. I must have made a noise, because the radiology staff quickly righted the table. I was unable to move for several seconds as my hearing slowly returned. I could barely comply when asked to roll onto the gurney so I could be transported to the CT room.  Hot tears rolled down my cheeks and pooled in my ears while I waited in the hallway outside the CT scanner, still gripping the useless tube of lavender, with my brain on fire. Scanned, I returned to my hospital room, still nearly senseless with pain but hopeful that the images would reveal the source of my leak.

The next day, we were dismayed and frustrated to learn that the CT scan had not identified the leak location. In retrospect, it’s not surprising that the CT images were inconclusive. The space around the spinal cord has no internal divisions or separations, so once dye is injected and begins to spill out of a large leak, it quickly travels along the whole length of the spine. The delay between dye injection and CT scanning allowed the dye to uniformly distribute along the spinal cord with no way to tell where it had initially leaked out. I later learned that standard myelography is consistently unhelpful for identifying the source of a fast leak.

We would have to go “blind.” In a blind epidural blood patch, blood from the patient is injected in a somewhat arbitrary location in the spine in the hope that it migrates to the correct spot to clot and seal the leak. The next day, I returned to the same radiology theater and received 30 ml of my own blood in my thoracic spine. Some of the same myelogram sensations returned during the patch, but with no dye or tilting necessary, thankfully no excruciating head pain.

Discharged from the hospital the next day, I followed post-patch precautions for six weeks (no bending or twisting and limiting lifting to 5 pounds or less), hoping and praying that I’d be “sealed and healed.” A gallon of milk and a full basket of laundry both exceed the 5-pound limit, and I found myself frustrated and hamstrung by an expanded list of things I could no longer do. My husband started to take on the look of the walking dead as he struggled to keep balls in the air both at work and at home.

When symptoms didn’t noticeably improve over the next few months, I saw more specialists. Some were sympathetic, some dismissive.  Without exception, their knowledge of CSF leaks was minimal. Each visit with a new specialist raised my hopes and then dashed them when it became clear, usually within the first few minutes, that I knew more about CSF leaks than they did.  Several implied that I was simply overstressed. One neurologist informed me that I was having tension headaches and should learn to relax.

“Download a meditation app on your phone,” she told me.

Another arrogantly dismissed me after he had exhausted his paltry knowledge of CSF leaks and had no suggestions for next steps. When I tried to reach him with questions, he refused to speak to me directly.

“We’ve done the maximum diagnostics,” I was told through his nurse.  Yet a cursory internet search would have pointed to cumulative improvement for leak patients with additional blind blood patching, at the very minimum. This was after he had already subjected me to unnecessary radiation exposure from a diagnostic I later learned was outdated and of limited utility for identifying CSF leak locations.

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Overcast and damp, spring arrived late that year even by Minnesota standards. Late-season storms left the ground snow-covered until mid-April, then brown, muddy and ugly for weeks afterwards. The weather mirrored my continuing symptoms, with both my life and the outside world at a complete standstill. It felt a little like a betrayal when the grass finally greened, trees leafed out, and lilacs bloomed while I continued to struggle.

I knew what I was feeling was not normal. As a runner, I was intimately attuned to the physical sensations of my body. Something was still wrong. My headache had started to evolve, moderating slightly but seeping across all hours of the day, a classic development for chronic leakers. The dull ache of pain between my shoulder blades was constant and immediate upon waking, and my head felt pressurized, as though someone had their hands squeezed tightly around my neck. Fatigue was constant. Some new sensations joined the parade of bizarre symptoms. I developed a strange feeling of moisture in my ears, as though I had just stepped out of the shower but lasting all day. My vision was blurry, and it hurt to move my eyes from side to side. Worst of all, I could see and feel my hard-earned fitness evaporating from lack of exercise. I searched “deconditioning” but stopped clicking when the results were too depressing. I remember thinking upon waking in the morning that the best part of the day, the part before I got up, was already over.

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Around this time, I stumbled onto an online patient resource site, Inspire.com. I was amazed to find that Inspire had a support community for spinal CSF leakers. Suddenly, I had a virtual roomful of fellow sufferers in various stages of the disease to ask questions, share frustrations and fears, and vent. As we exchanged stories, the worst part of this disorder began to become clear. Utter uncertainty. Crawl into an urgent care with severe abdominal pain and you’ll have a diagnosis of kidney stones and a clear course of treatment within a few hours. Not so with CSF leaks. Patients can spend months or years suffering with symptoms and seeking a diagnosis from doctors whose responses range from benign head scratching to dismissive contempt.

Once you’ve cleared the hurdle of diagnosis there’s often a lack of consensus about how to treat, leaving decisions about which course to follow ultimately up to the patient. After bed rest, blood patches are usually the first line of approach with weeks of uncertainty afterward as patients wait to see if symptoms improve. If multiple patches fail, surgery is the next option with even higher stakes.  Many leakers experience cumulative improvement from multiple patches, so the decision to proceed with surgery or pursue another patch is fraught with uncertainty as well.

Surgery to repair a leak is no minor thing. Neurosurgeons must cut through back muscles and permanently remove several levels of lamina, the pointy bones on the back of the spine. Once the bones are out of the way, they cut ligaments and connective tissue to reach the spinal cord, a glistening silvery-white tube about the diameter of a dime that normally lies inside a protective cage of bone. For anterior leaks from a tear in the front of the spinal cord (on the chest side), they have to gently manipulate the spinal cord to the side to locate and repair the hole underneath. Your spinal cord is not something that you want “gently manipulated.” If you’re picturing a smooth tube from top to bottom, add 31 pairs of spinal nerves branching from the spinal cord, each with its own feathery attachments that split into nerves controlling muscles and organs and sending information back to the brain. If a single nerve is accidentally cut, some muscle, organ, or patch of skin will no longer be able to send and receive messages from the brain. Surgery patients have the crushing uncertainty about whether their leak will be found and repaired, as well as the fear of trading disabling headaches for some unanticipated disability, paralysis, or stroke. Cutting through postural muscles means that recuperation time for spinal surgeries is measured in months, with full recovery taking up to a year; far longer than abdominal and even open-heart surgeries. And tragically, some leakers discover that their leak is related to a previously undiagnosed connective tissue disorder which leaves their spinal tissues fragile and prone to leaking again.

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Because I could still force myself to function for a few hours each day, the reality of my situation was mostly hidden from people around me. The symptoms of a spinal CSF leak are utterly invisible. Coworkers and casual acquaintances who knew what I was grappling with would try to encourage me with, “You look great, though!” not understanding how desperate I was. How filled with despair. A broken leg with a cast garners automatic sympathy. A headache? “Take a Tylenol and get over it,” was the undertone of all too many interactions. Incredibly isolating, I felt that only my husband and a few precious others who endured my daily phone calls and texts really understood what I was struggling with. People I would have expected to be present and supportive in many cases were painfully absent. It made sense to me when a fellow sufferer on the Inspire forum shared that, having dealt with both cancer and a CSF leak, she’d take cancer any day. People understand cancer. They get the gravity of it, the time frame of treatment and the uncertainty. When people hear “cancer,” the wheels of support immediately begin to turn and people spring to action. When people hear “CSF leak,” they say, “CS what?” There’s no simple way to communicate what’s wrong with you. It takes an explanation. And most people don’t have time for an explanation.

In my research that spring, I came across the Spinal CSF Leak Foundation website (spinalcsfleak.org) with its vast resources for CSF leak sufferers and discovered that there are four facilities in the U.S. that specialize in treating patients with leaks: Cedars-Sinai Medical Center in Los Angeles, Stanford University in California, Duke University in North Carolina, and the Mayo Clinic in Rochester, Minnesota. All were a cross-country flight except for the Mayo Clinic, a short 90-minute drive and in-network for our health insurance. Easy decision.

In late April, I began the time-consuming process of getting an appointment with Mayo Clinic's neurology department CSF leak specialists by requesting an appointment online, orchestrating a referral from my primary care clinic, making countless phone calls, and filling out endless authorization forms to transfer records from the multiple facilities where I had been treated over the last few months.

I accomplished all of this without any assistance from primary care clinic doctors or specialists while suffering through the fog of headaches and leak symptoms. One irony of our health care system is that patients are forced to navigate its ridiculous complexity precisely when they are compromised by illness and least capable of doing so. Because of the stunning lack of knowledge about this disorder, even among specialists, CSF leakers have to be masters of self-advocacy.  Many times I felt like Sisyphus, pushing the rock up the hill only to have the bureaucracy of the medical system shove the rock back down.

The Mayo Clinic finally called with the news that I had been accepted as a patient and was scheduled for the earliest available appointment — nearly two months out. With no other choice, I continued trying to manage symptoms while working and functioning nominally as a parent and spouse.  Our eldest daughter graduated from high school and I participated, at least marginally, in most of the festivities. I staggered through the last days of teaching. Plans for summer house-repair projects were cast aside, our usual summer garden went to weeds, and annual traditions of strawberry picking and jam-making were postponed for another year. With heavy hearts, we cancelled a long-planned family camping trip to Banff National Park in order to make time for the upcoming care at the Mayo Clinic. We knew that with summer jobs needing to take precedence, our eldest may not be able to join us next time, if there was a next time. Having already missed our middle daughter’s confirmation ceremony during a week of neurologist-ordered bed rest, it felt like I was handing over increasingly large chunks of my life to this thing as it demanded one sacrifice after another.

We planned to host a graduation party for our daughter at our house a day before my Mayo Clinic appointments, an almost Herculean task given my condition. With help from friends and family, we somehow managed to clean, decorate, and host a party for 60 people in spite of my limited hours of “up time.” That night, as I picked up half-eaten plates of food and put away the badminton rackets, I felt joy and relief at having pulled off this milestone event mixed with equal parts fear and worry about the next day’s appointments.

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"The Mayo Clinic, Rochester, Minnesota" by Vin 
Crosbie is licensed under CC BY-ND 2.0 

The Mayo Clinic’s reputation as efficient and patient-centered is well-deserved. Having worked as a research technician in a Mayo Clinic hypertension research lab in the 1990s, I was familiar with the Mayo model. A complex machine with thousands of moving parts, the Mayo Clinic excels at moving patients efficiently through departments and coordinating care among physicians to provide holistic care. But, like all health care, the quality ultimately rests on human interactions. In that we were not disappointed. Our first appointment with neurologist Dr. Jeremy Cutsforth-Gregory could not have been more of a contrast with our interactions with other clinicians that spring and summer. The first thing he did was ask me to tell my story.  He listened. Really listened. By itself, that was worth the trip. But refreshingly, incredibly, he actually understood CSF leaks. Dr. Cutsforth-Gregory is one of a small subset of doctors working in neurology, radiology, and neurosurgery at the Mayo Clinic who specialize in the treatment of CSF leaks. I had finally found my way to someone who could help.

Dr. Jeremy, as I called him, ordered yet another series of MRIs and we made our way through the maze of buildings and below-ground walkways to the imaging department. Not claustrophobic before this experience, I had begun to dread the hours spent inside that big white tube with the raucous, clanking noises that came to represent the sounds of my leak, the need to remain so still that you can’t even swallow, which only makes you want to swallow more.  At some point during the two-hour procedure, I tried to calculate the number of hours spent in an MRI over the last months. Eight? Ten? Twelve?  I lost count. The results of the MRI would be ready for our second appointment with Dr. Jeremy later in the day.

Finally read by a radiologist familiar with CSF leaks, the MRI results were definitive. I had extensive fluid outside of the dura for almost the entire length of my spine. A fast leak. But one that held the promise of being easy to locate, according to Dr. Jeremy.

Tears of relief welled up as he scrolled through the MRI images on the exam room computer to show us evidence of the leak. I hadn’t processed until that moment just how much I needed visual proof that what I had been suffering through was real. Looking back through my previous scans, he could also see signs of a leak that had been overlooked by doctors and radiologists in the Twin Cities.

To locate the leak site, he scheduled me for a dynamic myelogram a few weeks later. Similar to a standard myelogram, contrast dye is injected into the lumbar spine. The critical difference is that in a dynamic myelogram, real-time CT images capture the dye’s progress as it travels so that the leak site can be identified before the dye has a chance to distribute along the length of the spine. In practice, this means being on all fours with your rear end hoisted in the air and supported by a large foam wedge, shoulders down and arms stretched painfully over your head while a radiologist uses a large needle to inject dye into the lumbar spine. As with a standard myelogram, patients have to be awake during the procedure in order to be able provide feedback about sensations, with the added requirement of breath-holding so that motion-free images can be captured. With a normal spine, the radiologist would see a bright line of dye tracing the bottom of the spinal canal. With a leak, this bright line splits into a second, parallel line in the place where the dye leaks out of the dura.

After the terror and excruciating pain of my first myelogram, I dreaded a repeat. This time, I insisted on a preoperative sedative. Whether due to the Valium or the expertise of the neuroradiology team, the procedure was far less painful than my first. Before I even left the CT room, the middle-aged radiologist with a sweet Irish brogue told me he’d found it. A clear leak in the front of my spinal cord at T10-11 in the middle of my back, right at the location of the chiropractic adjustment. Again, I cried with relief. Identifying the location multiplied the chances of being able to target the leak with a blood patch.

In mid-July, I endured a second blood patch, riskier than the first in that it required a transforaminal approach.  With this technique, needles are inserted at an angle from both sides of the spine, guided by CT, to try to target the leak site at the front of the spinal cord. If blood is accidentally injected into one of the nearby spinal arteries instead of the epidural space, it results in an instantaneous and likely lethal stroke. The neuroradiologist was honest about the risks, taking time for a last-minute phone conference with the neurologist before the procedure. His words for the possibility of an adverse outcome: “catastrophic” and a “career ender.” I was acutely aware that it would mean much more than that for me.

I steeled myself for yet another procedure. Another surgical theater, face-down on the table with the disembodied voices of medical staff moving around and above me. Thanks to an amnesiac cocktail of Versed and Fentanyl, the hour and a half procedure flew by and I found myself back in the prep area, listening to the doctor reassure my grateful husband that the procedure went without incident. We returned home to wait with the by-now-familiar restrictions: no bending, lifting, twisting or straining. Patients are told to avoid sneezing and coughing. Nothing that could increase pressure and cause the patch to “blow.”

It goes without saying that vomiting is a bad idea. Unfortunately, when blood is injected into the epidural space, the sudden increase in volume can lead to a temporary state of high pressure. High pressure results in intense, excruciating headaches. Nausea. Vomiting.

The day after the procedure, I awoke with a piercing headache and a hint of nausea. “Please, no,” I remember thinking.  I tried talking myself out of it until it swelled into a terrible certainty. Intense, excruciating headache. Nausea. Vomiting. And vomiting again. My parents arrived, taking turns holding my hand and rotating ice packs on my forehead as I vomited repeatedly.  We all worried that I had blown the patch. Only time would tell.

Dr. Jeremy told me that the soreness should lessen a few weeks after the patch and that I should try to resume “normal” activity.  If symptoms persisted, it would be time to move to surgery for a more durable fix. I tried to convince myself that the patch had worked, but by early August, continuing headache, stiff neck, pain in my chest and between my shoulder blades told me I was still leaking. Yet another MRI in Rochester confirmed fluid outside the covering of the spinal cord. Dr. Jeremy had given me the option of trying another patch first, but was frank about the likelihood of patch success with anterior leaks like mine. Because of the difficulty of reaching the leak site in front of the spinal cord, they are less likely to work. With the school year already looming and teacher workshops in less than two weeks, I felt the crunch of time knowing that recovery from either patch or surgery was bound to take weeks. Reluctantly, I scheduled surgery for the soonest available date.

In the weeks that followed, I tormented myself with illustrations of spinal cord anatomy and YouTube videos of spinal surgery, researching the parts of the body served by the T-10/11 spinal nerves and trying to anticipate what I would lose in sensation or function if nerves were accidentally severed during surgery. I fixated on the possible risks including death, paralysis, and loss of bladder, bowel and sexual function, going back and forth about the decision on a minute-by-minute basis. I scrutinized my symptoms, trying to assess if my degree of current function was worth the potential risks of surgery. If I was functioning at 60 percent (with an admittedly reduced quality of life), should I risk that for the pain and months of recovery for surgery with an uncertain outcome? I tried in vain to recast my perception of surgery as something other than a frightening violation.

Parker's Lake Trail, Plymouth MN

Dr. Jeremy had cleared me for moderate exercise a few weeks after my July patch, so I walked incessantly, ruminating on the upcoming surgery. The stress of the last eight months was coming to a head for me. I wasn’t sleeping and I couldn’t force myself to eat. I would wake at 3 a.m. to defrost the freezer, obsessively clean a bathroom, or walk. I walked and walked. It was the only thing that helped to alleviate some of the anxiety. My son, 14, would gamely join me on many of these long walks around a nearby lake, listening and reassuring with the wisdom of someone twice his age. I walked 5, 6, 7 miles a day. Knowing I wasn’t likely to make anything worse, I even went on a few “rage runs,” my term for a run of frustration and desperation. Fit and healthy at 118 pounds in January 2018, by mid-August I was down to 104 pounds, frail and frantic. I needed a solution.

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"Stained Glass, Saint Mary's Hospital,
Rochester, MN" by Tadson is
licensed under CC BY-ND 2.0 

Surgery day.  We arrived at St. Mary's hospital in Rochester at 5:30 a.m., surprised by a line 50 people deep of surgical patients and family members waiting to check in. I considered bolting for the nearest exit. Instead, we all shuffled obediently toward the desk, exchanging sympathetic glances, each of us silently carrying our fears along with stories of illness and injury. I couldn’t help thinking of that Bible verse about sheep being led to slaughter.  We finally checked in and were led through various surgical staging areas, losing bits of myself along the way until we arrived at the last stop with me, naked and terrified inside a paper gown, Bob standing nervously alongside me with my belongings dangling from his wrists in plastic bags. When I kissed him goodbye and they wheeled me into the cavernous operating room, cold and bright and bustling with medical staff, I could no longer hold back the tears. The last thing I remember is a kind anesthesiologist saying “Let’s give her something to make her feel better.”

Our kids drove down from the Twin Cities that morning to wait with Bob and see me afterwards. To deal with her nervousness while I was in the operating room, our middle daughter did a training run through Rochester’s sunny streets and parks near the campus. Bob tried unsuccessfully to busy himself with work on his laptop, resorting to texting family and waiting for updates.

Surgery took five and a half hours. I awoke, moaning, in gut-wrenching, indescribable pain, every muscle in my back and torso spasming from the 6-inch incision and more than 100 continuous stitches in my back. “Did you find the leak and did you fix it?” I managed to ask. Yes to both, thank God. The surgeon told my husband that the tear had been roughly a centimeter in length, far too big to be sealed with a patch. I had made the right decision. I had also been very fortunate. While I experienced some weakness in my right leg from some nerve irritation during surgery, this resolved over the next few days. I could move. I could feel all my extremities. And in spite of punctures from stabilizing pins placed in my skull during surgery, my head felt clear for the first time in months. The worst was behind me. I spent four days in the hospital, my husband there with me every second, before being discharged to begin the slow and painful process of recovery. The day after I was discharged, still groggy with pain medications and anesthesia, I said a tearful goodbye to our firstborn as the rest of the family packed the car to take her to college without me.

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In medicine, there are clear procedures for reporting adverse outcomes. Hospital accreditation commissions and state boards have guidelines and reporting mechanisms requiring physicians and hospitals to track medical mistakes and bad outcomes of all kinds. There is even an online, FDA-sponsored portal (MedWatch) for individual patient reports of medical errors. Not so for alternative medicine, including chiropractic. Aside from licensing and regulatory oversight, there is no state or national organizing body for reporting bad outcomes. A 2009 systematic review found a grand total of one randomized controlled trial evaluating adverse effects of chiropractic since 1966. Touted as safe, there is actually no clear data showing how often patients are seriously harmed by chiropractic manipulation. Medical staff who treated me openly acknowledged that they regularly see patients who come to them after chiropractic injury, and there are documented cases in the literature of chiropractic adjustments causing CSF leak.

The chiropractic industry relies on voluntary self-reporting of injuries and malpractice lawsuits to police providers. However, it is extremely difficult to find a lawyer willing to take a chiropractic malpractice case, thanks to pre-treatment waivers and the high bar of showing that the chiropractor’s actions fell outside the bounds of the “standard of care.” One lawyer I consulted told me I would have needed to have an MRI before my chiropractic treatment and immediately afterwards in order to have a hope of proving that the injury was the result of the chiropractic manipulation. Not too many people insist on a pre-treatment MRI, just in case they need to have recourse against their chiropractor.

There is no doubt in my mind that my chiropractic adjustment was responsible for my dural tear and CSF leak. I don’t exhibit signs of a connective tissue disorder that would predispose me to a leak and the surgeon looked for a bone spur that could have contributed to a leak during my surgery but found none. At the very least, my experience should serve as a cautionary tale for others hoping to find relief with chiropractic. While many people swear by the results of chiropractic manipulation, there are very real and potentially severe risks when someone carries out high velocity spinal adjustments like the one I experienced.

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Once home, I couldn’t sit up, get out of bed, brush my hair, get dressed or shower without help for weeks. The pain was intense, and I can’t imagine what I would have done without my family’s help and the miracle of narcotics. I didn’t need a clock to tell that it was time to take more meds; I would start shaking from pain when the Oxycodone began to wear off. One of the first evenings home, we tried to watch a TV comedy for distraction until the pain turned my laughter into tears. I moved in a circuit from the bed to the couch and back again with a constant rotation of ice packs on my back.

With my daughter at the Minneapolis Turkey Day 5K 2018

In the early weeks, I forced myself to walk in short, one-block loops around our neighborhood, slowly and painfully at first and clutching the arms of whoever walked beside me: my husband, kids, mom, sisters and close friends. Milestones included weaning off narcotic pain medications, graduating to solo walks of increasing distance, rolling over in bed without gritting my teeth in pain, returning to the classroom six weeks after surgery, starting real sweat-producing workouts at the two-month mark, walking a 5K turkey trot with my daughter who was home on break, and a three-month follow-up MRI that showed — thank God — no more fluid outside the dura. It is still shocking to see images of my spine without several levels of bones that used to shield the back of my spinal cord.

Eight months out from surgery, I can finally bend over to put on socks and shoes without the anticipation of pain. Physical therapy has helped me gradually regain nearly all my strength and flexibility, although the incision and muscles surrounding it still feel stiff and strange. My back still gets fatigued from too much standing. But my head feels blessedly clear, the blurred vision and ringing in my ears having mostly faded about a month after surgery. I have the ghosts of a few leak-type symptoms that keep me terrified of a continued leak: a slight ache between the shoulder blades, a strange sense of heaviness in the center of my chest, both aggravated by cold and more noticeable late in the day. I’m hopeful that these are the last vestiges of nerve damage that will dissipate with time.

I still descend into panic at the slightest sign of headache, and fears of a relapse are real and significant. No one can guarantee that I will not leak again. There are no long-term follow-up studies of CSF leak patients and there are plenty of people for whom surgery (or multiple surgeries) does not provide a durable fix. In the background of my daily life lurks the constant awareness that my health and quality of life could once again evaporate if I should start leaking again.

Next to heart surgery, spine surgery patients have the highest rates of post-surgical PTSD and depression, perhaps because of the trauma and fear surrounding surgery on a part of the body so integral to survival. I recognize some of the signs of fixation in myself. In the weeks after surgery, I spent an inordinate amount of time trying to figure out what happened during the five and a half hours that I was unconscious. I watched videos of the intubation process, scoured my own surgical reports for details, and pored over X-rays taken during the procedure with incongruous silhouettes of surgical instruments overlying, or perhaps within, the incision on my back. I contemplated buying a spine surgery textbook on eBay for a more comprehensive account of the surgery process and watched an entire two-hour manufacturer’s video training series for the surgical table used during my procedure so I could picture how I was moved and positioned. If someone had videotaped my surgery, I would’ve eagerly watched the whole five and a half hours (probably multiple times) to satisfy my obsessive need to know.  I recognize that I may still have work to do to process this experience.

I have started to reclaim some normalcy in my life. Hopefully a new, better normal. I have learned that I need to approach life differently, maybe reframe what it means to be selfish.  I used to scoff at meditation as a waste of precious time. Now, I see it as an important daily practice. Prayer that used to feel forced and mechanical now feels much more like an intimate conversation. I've gained a deep compassion for others who are suffering regardless of the cause.

I don’t harbor anger or resentment for the chiropractor. I know she did not intend to do me any harm and that focusing on the injury will only slow my healing. But I do want to prevent others from having to endure this ordeal. To this end, I wrote to her, asking her to consult the literature showing a connection between chiropractic and CSF leaks and to reassess the degree of force she uses in thoracic adjustments.

I suspect that there are hundreds or possibly thousands of people out there, undiagnosed, desperate and disabled by a CSF leak. I remain active on the patient support forum to provide support and hope for those who are still suffering.  If I can bring awareness to this disorder and help even one person recognize symptoms, advocate for themselves to get a quicker diagnosis and prompt treatment, my experience will have been worth it.

I am clawing my way back to fitness at age 50, exercising an hour a day, six days a week again. Biking, aqua-jogging, strength training. And I’ve started running, slowly, gently, easing back in. I’m considering a race this summer, if only to mark the end of the ordeal. At the same time I’m afraid of jinxing my recovery by officially pronouncing it “over.” I am trying to stop qualifying my improvement with statements like, “for now” and “so far.”

There are some things I may never do again out of fear of wrenching my spine and re-leaking: downhill skiing, sledding, trampolines, roller coasters, even playful wrestling with my kids.  I can live without these things, but I mourn the loss of my invincibility.  I hope the day will come when I am free from the specter of leaking again and the nagging fear that I am not yet healed. I wonder if I will ever be able to fully trust my body again.

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Mostly, these days, I feel lucky. So incredibly lucky. I am lucky that I had the serendipity of background knowledge to help me self-diagnose early. Lucky that my symptoms were not as debilitating as for others, lucky that I had the energy and resources to advocate for myself, lucky that the Mayo Clinic was geographically close and in-network, lucky that my neurosurgeon was skilled and able to find and repair my leak. Lucky that I was surrounded by people who stepped in to care for me when I needed them, my sweet husband, my kids, parents, sisters, in-laws, friends.

I am relearning how to plan for the future again now that I’ve emerged from the rabbit hole of my CSF leak. Our camping trip to Banff in the Canadian Rockies is back on the calendar for this summer.  I’m picturing running along the shores of a glacial lake, surrounded by my family, breathing in the fresh mountain air with my head buoyant and light, doing my best to allow myself to feel healthy and whole again.

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Epilogue: July 2019

Our family just returned from a fantastic trip to Banff, Kootenay, and Jasper National Parks in the Canadian Rockies. I feel so blessed to have been able to make this trip together. Our college-aged daughter was able to take time away from three summer jobs to join us for two weeks of camping, hiking, laughing and family time. As the one-year anniversary of my surgery approaches, I am trying to take stock and stay grateful. I'm still afraid of a recurrence, still afraid of what could loom around the next corner, but as many have told me that it would, the intrusion of anxieties into my thoughts is less frequent. I sometimes go several days without thinking about my health challenges. Time truly heals. And that's all I can ask.