Your life can change in an instant. You may not immediately realize the breadth of the change or the full range of its implications, but you’ll always remember when the change happened.
Mine changed one morning on a beautiful mountain trail. It was August 2003.
I fell. Hard.
And as a result, my brain no longer floats properly.
That fateful fall – coupled with an unknown pre-existing neurological defect – led to tiny perforations in my spinal column, allowing my cerebral spinal fluid (CSF) to leak out a tad faster than my body can regenerate it. With insufficient CSF, my brain settles in my cranium, putting pressure on various nerves. The medical term: spontaneous intracranial hypotension (SIH).
My snarky term when symptoms are bad: I’m a quart low.
It’s a hidden disability. Looking at me, you’d never know.
Yet this condition has changed my life in profound ways. There are the myriad internal physical symptoms, and then there are unexpected emotional ones. I am a different person now; someone I often don’t like and would rather not hang out with. Someone who sleeps too much, can be short-tempered and cranky, needs a daily nap, and suddenly has low tolerance for loud noises, inconsiderate people, incessant chatter, and typically stressful situations such as traffic and office environments.
Ugh. I’ve learned to compensate so that no one knows unless I tell them. Even then, they can’t really understand because it’s all going on inside me. Mostly, I bite my tongue and hide my discomfort.
And, for extra challenge and an element of surprise, my symptoms wax and wane. I never know how I’m going to feel from one day to the next. If I’m experiencing a spate of days with light symptoms, I don’t know how long that might last. Needless to say, I avoid making long-term plans.
The underlying congenital condition that causes my woes today (spina bifida occulta) wasn’t an issue when I was a child, beyond vague memories of the occasional “migraine” headache that I seemed to outgrow, slight scoliosis, and the patch of fine hair that grows on the small of my back, at the base of my spine.
For years after my diagnosis of SIH in 2004 I refused to even consider myself as having a disability. I always hoped I would spontaneously heal and return to normal. While that did happen a couple times in the early years, another trip, fall, or accidental hyperextension of my spine would create a new leak. Eventually, the leaks and their symptoms became chronic. I had to admit that I likely never will return to my old normal, that this is my new normal. I came “out” as disabled a couple years ago. It makes cancelling plans at the last minute much easier.
It All Started So Innocently
I’m running along a single-track dirt trail high in the Cascade Mountains of Washington state, my boyfriend Mike and my two Alaskan Malamutes, Maia and Meadow, keeping me company. It’s the last weekend of August 2003.
Mike and I volunteered to remove trail markings – flagging – while following and monitoring the last place runners over the last 20 miles of an ultra-distance race, a hundred-miler called the Cascade Crest Classic. It’s mid-day, sunny and warm and the views we’re treated to along the way have us mesmerized – sharp mountain peaks skirted in dense evergreen forests, lakes, streams, and in the distance, snow-capped Mount Rainier floating above every other landmark in a blue sky that stretches forever.
As we steadily move along the trail, untying flagging from tree branches and shrubs, we engage in our usual, easy banter; we’ve been together eleven years and we’ve spent countless hours together running trails. We’ve done this job before and come back each year because of the course’s beauty, and because we get to cover 20 miles at a relaxing pace with my dogs while enjoying the food and beverages provided the runners at aid stations every five or so miles – less for us to carry. Sometimes we get to play a role in urging a very tired, exhausted runner at the back of pack who’s facing a time cut-off at the next aid station and might be pulled from the race oh-so-close-to-the-finish to keep moving, one foot in front of the other, pushing them gently toward and across the magical finish line.
We listen to the birds and watch the dogs trot along the trail until they catch a scent and follow it a few feet off trail before loping to catch and pass us to resume the lead. I’m 46 and this is my nirvana, running trails with the people I love and consider my family – Mike and the girls, and more broadly, the trail running community and the people who support them at races.
Following the girls, sharing funny anecdotes with Mike while running just a few feet in front of him, I suddenly catch the toe of my right foot as my left foot is swinging forward; my upper body uses that momentum to try to fly forward while my right foot keeps me tethered to the ground, causing me to hit the ground hard without time for my hands to break my fall. I feel my spine hyperextend just before I hit the dirt, first with my chest, then the side of my head. The wind is knocked out of my lungs and it seems like forever before I can inhale. I don’t move; I’m doing a quick mental tally of limbs and joints, making sure nothing’s broken or seriously tweaked.
“You okay?” Mike asks with alarm while the girls come back to sniff and kiss me; they don’t often see me laying on the ground and are concerned. Trail running involves falling, especially in the mountains of the Pacific Northwest where there are rocks and roots everywhere just waiting to catch your toe or twist your ankle. Mike and I have watched each other take spills over the years, and usually it’s our ego suffering the worst damage, but he can tell this fall was harder than most.
“I’m fine,” I say even though I’m not totally convinced. I get up, dust myself off, and am thankful it wasn’t worse – no broken bones or skin, no stretched or torn tendons to interfere with being able to finish the job of sweeping the trail. We’re a long way from help and I don’t want to become a burden to Mike or the race organization. I start walking, slowly and carefully to make sure everything’s working, Mike following me with concern until I start jogging again. I’m sore – right upper arm, right hip and thigh which took the brunt of the impact – but it’s tolerable and feels better the more I move. Within minutes we fall back into our usual trail-sweeping rhythm of running several yards, stopping to pull ribbons, running some more and pulling more ribbons while smiling at the girls’ antics and entertaining each other with stories and jokes as we keep the last place runner in sight without getting annoyingly close.
Something’s Not Right
By mid-September I’ve got a constant headache that will not go away or respond to ibuprofen. Otherwise I feel fine and keep running almost daily.
Soon I begin noticing that when running down steep trails my headache is much worse, almost debilitating with each forceful footfall as if my brain will explode through my scull right behind my eyes. I can hardly carry on a conversation – with my dogs or whomever I’m running with – until the terrain levels or we go uphill and the pressure lessens. I go on vacation to a dude ranch in British Columbia, a place my dogs and I have enjoyed many times previously. This trip, though, every time my horse trots, I think my scull will break, the pressure inside so intense, like my brain is a bowling ball knocking against the inside of my scull with every bounce in the saddle.
The headaches never go away, even when going about normal daily routines; they’re chronic. Except when I sleep; suddenly I’m sleeping really soundly and dreaming, a lot.
In mid-September I make an appointment to see a doctor and am seen by a P.A. I try to describe my symptoms – intense pressure behind my eyes and nose, worse when I run and especially run downhill. He suspects sinus infection, prescribes an antibiotic and instructs me to call if I’m not better in two weeks.
I take the antibiotic. I don’t feel better. In fact, the headaches get worse. Despite the horrible pain when I run – and oh god, the downhills are so bad that I walk them now – I keep running because that’s the thing that keeps me sane. After two weeks I call the doctor and this time I see my primary care physician. It’s late September. She listens to my symptoms and refers me to a neurologist. I call to make that appointment; the earliest I can get in is early December.
Searching for Answers
My road to diagnosis is long and fraught, in large part because this condition is rare and difficult to diagnose. Back in 2003, few physicians had any experience with diagnosing it, and that’s only slightly better today although recently a Spinal CSF Leak Foundation has been created to help patients and promote learning and recognition of this rare condition within the medical community. And while there are treatments (often surgical) that can help many with this condition, my exceedingly rare tethered spinal cord makes them too risky for me. So, by necessity I’ve learned to adapt and live with my disability.
I keep running, even when it hurts my head, as is often the case. I keep doing the things that bring me joy, always knowing that at some point my condition could get much worse. I live for today, which explains a lot of my life choices.
And I share my experience, in case it might help others. The following article was published by the Mayo Clinic in a series of columns written by patients or caregivers with the goal of helping health care professionals be more aware and understanding of their patients’ experiences. (I learned a few weeks ago that my piece will be included in an anthology of articles from 2019.)
My Leaky Life: Learning to Live with Chronic CSF Leaks
(Published in the Mayo Clinic/Inspire Series: Experts by Experience column, April 2019)
My dogs and I prepare to head out for a walk in the forest. They’re excited, which makes it challenging for me to put their visibility vests on them. My anger instantly swells out of proportion. I growl, move abruptly. At the instant I’m short-tempered with them, I simultaneously know it and back down, asking myself, “Who the hell are you and what did you do with Rebecca?”
This ability to analyze my behavior in the moment is the result of too much practice. Over the past fifteen years, I’ve had to learn to control a temper I never used to have, a temper that’s the by-product of a condition that for most of my life I also didn’t know I had.
Fighting for a Diagnosis. My twin birth defects of spina bifida occulta and a tethered spinal cord were discovered following a hard fall while running trails in 2003. It took nine months, three neurologists, a spinal tap, two MRIs, and dogged persistence on my part to receive an accurate diagnosis for my horrible, months-long headaches, tinnitus, dizziness, fatigue, and brain fog: spontaneous intracranial hypotension caused by leakage of cerebral spinal fluid (CSF).
Because I kept running when I could, the first two neurologists didn’t believe my headaches were bad enough to fit the CSF leak diagnosis; most patients can’t tolerate being upright, let alone run. It was the third neurologist – a former endurance trail runner himself – who understood that running was my coping mechanism and that as an ultra-distance trail runner, I had high pain tolerance.
Coping. After many cycles of CSF leaks – which I jokingly refer to as being a quart low – I can match the degree of the leak to personality changes that weren’t initially obvious to me. My emotional reactions were something new. I was easily irritated, to a greater degree and much more quickly, by people I knew as well as total strangers, by loud noises, traffic, computer glitches … basically, by normal life. Where before that fall in 2003 I was upbeat, with boundless energy for friends, work, activities, even chores, I now felt unmotivated and easily angered.
I’ve learned to anticipate the bad times as well as situations (and people) that are triggers. If unable to avoid them altogether, I stop my reaction pre- or mid-outburst with a mental “Chill!” that reminds me that the problem is me, not them. But it makes “fitting in” with cultural norms regarding work schedules and socializing challenging. I berate myself for not getting enough done, for sleeping too much, avoiding people, for … not being the me I was before CSF leaks. Cutting myself enough slack to rest and heal with each new leak is tough.
I keep running, always with my dogs (my emotional therapists). The pounding is sometimes excruciating inside my head, but I run – gently, less far, no longer racing – because it helps me cope. That runner’s high and sense of well-being are worth the extra pain. Running trails in the forest among trees, streams, wildflowers and wildlife, moving – no matter how slowly – keeps me sane. In those moments, I don’t feel disabled; I don’t think about pain. My creativity flows and I feel normal for a while.
Lifestyle Changes. Almost unconsciously, I’ve crafted a lifestyle that accommodates my condition, one that puzzles family and friends. Abandoning a twenty-year legal career, in 2005 I left Seattle to live on five acres in rural Idaho. I knew I needed space and quiet to escape having to cope with too-close neighbors or typical urban cacophony.
I left cushy jobs with good benefits because dealing with co-workers, work schedules, and commuting was almost unbearable. I’m now self-employed, working from home, in charge of my own schedule, which reflects how I feel. Most days, I nap when “normal” people are taking a lunch break, which allows my CSF levels to recoup. Rest and caffeine are the only treatment options that help. I shun most social situations, especially big, noisy gatherings.
Today I’m honest and open about my condition, the chronic pain and its emotional impacts. I’m more Stoic: taking each day as it comes, grateful for its gifts, not dwelling on the former “healthy” me. My dogs help defuse the stress of chronic pain while joining me in a lifestyle that accommodates my disability and makes me happy.
I wish a medical professional had warned me what was ahead emotionally, that I hadn’t had to figure this all out on my own. Discovering the Spinal CSF Leak Foundation and the Inspire community of fellow CSF leakers this past year has been a godsend. Sharing stories, lessons, and (most of all) hope has helped me know that I’m not alone.
Well Hello! You’re Back!
The past couple of days I’ve enjoyed this out-of-body experience of noting that my old self, my pre-diagnosis “normal” personality, has made a reappearance.
It’s so strange, like I’m one Rebecca watching the other Rebecca. With that same ability to step back and recognize my evil-twin cranky persona in the moment she’s being short-tempered that I described in the Mayo piece, I can see – and feel – that my non-leaking happy personality has returned. I haven’t seen this Rebecca in ages.
I’m saying sweet nothings to my dogs, even when they do something that, when I’m dwelling in the darkness of my worst symptoms, annoys me and makes me sometimes (to my everlasting shame) bark at them. I feel like Mr. Rogers has come for a visit, sharing his warmth and optimism. It’s a wonderful day in our neighborhood.
I’m getting work and chores done without procrastinating or waiting for energy and motivation to return. Even the legal cases I’m currently involved in can’t try my patience (and that’s saying a lot).
I’m feeling creative. I ordered a beginner’s kit of colored pencils and pastels so I can pursue something I’ve wanted to try for years: drawing.
I writing again.
I’d almost forgotten who this Rebecca is.
I’d feared she might never reappear.
It’s so fun to have her back.
I have no idea how long she’ll be here, but every day she is, is a gift.
Live your best life now. Today. Every day. Don’t wait.
[Big thank you to Susan Tasaki, friend and Senior Editor at Bark magazine, for editing the Mayo/Inspire piece for me. Susan, you rock; thanks for always making me sound smarter than I am.]
Photos: all taken the morning of January 17, 2020 while running on a freshly-groomed Forest Service road with my dogs, grateful for perfect conditions, both in weather and in body.