In early June 2024, I picked up my paddleboard, noting that it felt heavier than I’d remembered. Over the next few hours, I developed searing neck pain, cognitive changes, and profound dizziness. I lay on my bed, slipping in and out of awareness.
At the ER, a head CT was normal, and I was reassured that I was fine. Weeks passed in a haze, and I began to notice that a headache predictably set in within 10 minutes of getting up in the morning.
Although I am a doctor — a psychiatrist — I had no idea what had happened to me. It was only when I consulted ChatGPT that an answer seemed to materialize. I told my primary care physician that I thought I had a spinal cerebrospinal fluid (CSF) leak.
I recall learning very little about spinal CSF leak in medical school, and that seems not to have changed. In the last 17 months, I’ve been incredulous at the lack of awareness about the condition among most medical practitioners. I’ve largely had to direct my own care, with the help of fellow sufferers who share resources, tips, and support online.
“Almost every single patient has had to research their condition and be their own advocate,” says Jodi Ettenberg, board president of the Spinal CSF Leak Foundation, a nonprofit organization aimed at improving research and access to care for the condition.
In 2017, Ettenberg was a 38-year-old lawyer turned acclaimed food and travel writer who underwent a diagnostic lumbar puncture (LP) and suffered a life-changing spinal CSF leak.
Ettenberg later learned that the 18-gauge “cutting” needle used in her LP was inappropriately large, and she was not promptly treated. She did eventually get patched, but complications have deterred her from further treatment.
Ettenberg’s story is unfortunately not unusual. The most common cause by far of spinal CSF leak is iatrogenic, whether from a lumbar puncture, when the dura is accidentally pierced during an epidural in labor, or during spinal surgery.
In fact, Dr. Andrew Callen of the University of Colorado’s CSF Leak Program estimates that between these iatrogenic sources, doctors are causing at least 400,000 new chronic post-dural puncture headaches (PDPH) per year. He says that makes it a far more common, though less recognized, problem than Parkinson’s disease. While some of these leaks will resolve with conservative management, many will not.
We already have a means to prevent a significant number of PDPH cases. Use of an atraumatic, “pencil-point” needle, designed to subtly separate dural fibers instead of “cutting” through them, results in a much lower risk of procedural complications than a conventional needle. Nonetheless, Callen says there is likely not a single LP hospital kit in the country that contains a non-cutting needle. One expert in the field estimates that making this the standard of care could reduce iatrogenic spinal CSF leak by as much as 60%.
Spontaneous Intracranial Hypotension (SIH), a spinal CSF leak that occurs spontaneously, is considered rare, affecting 4/100,000 people. But doctors are now more frequently diagnosing a type of SIH, the CSF-venous fistula, an abnormal connection between the sub-arachnoid space and a paraspinal vein.
Spinal CSF leak often co-occurs with connective tissue diseases like Marfan’s or hypermobile Ehlers-Danlos Syndrome (hEDS). HEDS, which I have, also disproportionately affects women and is under-recognized by doctors.
Many doctors know only the classic syndrome of a postural, back-of-the-head headache. In fact, symptoms vary widely, and imaging findings can be subtle. The delays are costly; faster treatment leads to better outcomes.
There are few major leak centers, with long wait times. Difficult as my own situation is, I know I am lucky to have the means to travel for care — not everyone does.
Calling what I experience a “headache” is a misnomer. After a few minutes upright, a weight like an anvil builds in my head, forcing me into a horizontal position. I don’t go to movies or out to eat, and I don’t travel, except for appointments.
Not surprisingly, a 2023 study found that over 78% of SIH patients experience depression, and 64.2% have had suicidal ideation. On a spiritual well-being scale, SIH patients scored significantly worse than patients with AIDS and cancer.
As a physician, I empathize with the lack of familiarity; doctors only know what they know, and headache, after all, is a common complaint. But as a patient, I can’t help feeling angry. It seems that many doctors simply do not believe spinal CSF leak patients.
Says Callen, “It’s so sad to think that this is what somebody has to go through to get care.”
We can’t yet prevent spontaneous leaks like mine, but we can reduce iatrogenic cases and improve care for spinal CSF leak patients. Doctors performing spinal procedures must understand the potential for significant harm. First-line clinicians should recognize that not every spinal CSF leak is a textbook case and should know how to promptly refer patients. Above all, doctors should listen to their patients who tell them: “This is not just a headache!”
Did you learn much about spinal CSF leak in medical school? Share in the comments.
Susan Mahler, MD is in private practice in North Adams, MA and is also a writer. Her essays have appeared in The American Scholar, The Threepenny Review, STAT, and the Psychiatry at the Margins substack, among other places.
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