Earlier this month, the spinal CSF leak neighborhood misplaced a brilliant gentle when Connie Rim handed away. Her Facebook web page, known as My CSF Leak Story, was the place she detailed her lengthy battle attempting to get sealed and healed. Just a few days after she handed, her husband posted phrases from Connie about her dying, sharing it with the world.
Connie sustained her leak 5 years in the past throughout spinal surgical procedure, and tirelessly rallied her physique and thoughts in her makes an attempt to get care and discover her method again to a spot of much less ache. Like me, she discovered solace in sharing this journey publicly. In her case, by way of movies that she posted to Facebook and TikTookay, detailing her many debilitating signs, explaining the procedures and assessments she bought, and patiently answering questions.
When somebody is chronically unwell and takes a break from therapies, there’s inevitably a swell of individuals asking why. “Why aren’t you doing more?”, as if it’s as much as you to not be higher. As if there’s full management of the lengthy tightrope you’ll want to stroll on whenever you’re coping with a number of complicated circumstances. Despite her openness and uncooked, unflinching movies, folks nonetheless requested Connie this query.
Perhaps in response to this, or for different causes (I don’t know), in May 2023 Connie posted a protracted record of procedures and assessments and docs she’s seen proper earlier than she headed out to Mayo for extra leak diagnostics and procedures: “This cannot be my life. This is not a life. I’ve been fighting to get better and advocating for myself as much as possible but in truth, I’m tired now. Nothing is as easy as it once was,” she wrote then.
By this level, she had been to leak specialists and nonetheless her leak(s) weren’t sealed. She was in fixed ache. She reposted that record, up to date, a couple of days earlier than her dying.
She did get therapy after that May 2023 submit, one which introduced her ache down just a little bit. We may all really feel her pleasure and hope when it did. But that therapy failed, and when it failed it introduced a brand new, horrible set of signs. Since late January 2024, she skilled extreme stabbing ache from three to over twenty occasions every day, at what she labeled as a stage 10 ache. To be in ache all day, after which layer upon {that a} new and protracted, paralyzing ache… I can’t think about.
By mid-May, she was gone.
Reading between the strains of the message her husband posted, she hit a wall the place the excruciating ache she skilled second to second couldn’t be managed.
“Some days, I’ve been given 10-15 minutes of my precious baseline pain, and for that. I’m grateful. But during the remaining 23 hours and 45 minutes, I was always crying, fighting, and, at times, in so much pain that I could do anything but curl in a fetal position in darkness,” she wrote.
Her household and husband have been loving and sort, she had lots of help, and she or he had a neighborhood who rallied round her. I perceive that these wonderful issues is probably not sufficient when you find yourself trapped in a physique that tortures you.
Studies present that in a power state, ache can disrupt the communications between mind cells, resulting in a discount within the means to course of feelings — particularly detrimental feelings. Pain modifications your mind. I skilled a model of this myself not lengthy after my leak started: already in power ache for years however not but conscious of mast cell ailments, I ate a very excessive histamine meal of spaghetti and shared a glass of wine with my household. It was this meal throughout these preliminary leak days, earlier than I went to Duke for therapy, that tipped my physique its new, unruly state. After that meal, I had full-body burning and nerve ache 24/7. And it didn’t go away. It felt like I used to be being dipped in acid, with no respite from the torture.
It was a fellow affected person who urged me to consider MCAS, given my signs. I hadn’t heard of mast cell activation syndrome earlier than. With no docs to help me, I scrambled to get my mast cells beneath management and attempt to make it cease. During these weeks of absolute hell, till I discovered over-the-counter medicines that labored for me, I begged an in depth good friend to assist me discover a method out endlessly. (They declined, have been compassionate all through, and as an alternative tried to seek out me a grief therapist domestically. What helped on this case wasn’t remedy, it was discovering respite from the fire-burn throughout my physique due to antihistamines and a low-histamine food regimen.)
And that was how I felt with just a few weeks of torture.
Connie lived with that ache that for a few years. As she stated in her writing, “The pain determines my emotions, NOT the other way around (as some people believe).”
Her passing hit the leak world very arduous. Even those that didn’t know Connie personally usually watched her movies on TikTookay or Facebook, or interacted along with her on the teams. In my case, I used to be digital associates along with her for years and we exchanged audio messages about methods, instructed jokes, tried to maintain ourselves sane on this Groundhog Day world we lived in when staying sealed felt like a pipe dream.
And but, regardless of the ache and the exhaustion and the procedures, Connie remained joyful the place she may, she appreciated what love she had in her life, particularly her deep and delightful love for her husband, and noticed humour the place she was ready. She had tons of help. The ache decided her feelings, as she stated. She was a prisoner of her fixed agony.
I really feel offended for her and her household, and for all of us. In taking a look at that lengthy procedures record, it appears like she was failed so many occasions alongside the best way. Even as not too long ago as this 12 months, she offered to the ER and later shared a video explaining that the on name physician stated her signs have been psychosomatic. How are you able to take a look at that blisteringly lengthy record of therapies and assume it’s psychosomatic?! The ongoing nerve injury and tissue injury, and scar tissue that may entrap her nerves, all of it is sensible wanting on the lengthy record of what her physique had been by way of.
But no, she was instructed it was all in her head.
That’s a part of what leaves me rattled, for anybody with this situation or different invisible circumstances the place metrics for analysis and therapy aren’t minimize and dry. I’m engaged on the slides with the US and different spinal CSF leak foundations for the annual consciousness week for this situation, known as leakweek (which begins June third). In it, we share the various kinds of diagnostics and word that regular imaging doesn’t rule out a spinal CSF leak. Normal opening stress doesn’t rule out a spinal CSF leak. Normal something doesn’t rule it out; we’re trapped on this infinite loop of being unable to ‘prove’ quantitatively what we have now aside from signs, but usually we’re instructed we aren’t dependable narrators when sharing our signs.
And so we strive, we strive so arduous.
We current ourselves firmly however with out panic, however not TOO jokingly else we appear ‘not sick enough.’ We preserve spreadsheets, we share knowledge, we come armed with supporting research however not TOO many, else it looks as if we predict we all know an excessive amount of. Outside the few leak consultants on the market, who’re fortunately coaching docs usually, this dance continues. It’s exhausting to dwell in ache and to lose your mobility and a lot extra, however add the suspicion and disbelief to the combo and it drags your soul all the way down to the bottom.
Because regardless of the advocacy, regardless of the schooling, the myths persist.
Outside the leak consultants, sufferers report that docs nonetheless assume a lumbar puncture leak is “self-limiting” (i.e., that it goes away by itself — I want!). Many nonetheless doubt the existence of spontaneous intracranial hypotension (SIH), the place a leak can happen abruptly; this may be on account of a bone spur or calcified disc gnawing into the dura til it tears, or a coughing match, or lifting heavy weights, or one thing else being the final straw for a weak point within the dura mater the affected person wasn’t conscious of. And a extra recently-discovered kind of leak, a CSF-venous fistula, may be elusive on current imaging varieties, although a more recent photon counting CT machine is recognizing them extra regularly. Canada doesn’t have one, and the US solely has a couple of.
The brutality of this situation is difficult to precisely put into phrases. Not solely the ache itself however the uncertainty and uphill battle in getting care. It’s generally misdiagnosed and under-diagnosed. The psychological well being burden can also be important: a high quality of life research in spinal CSF leak sufferers from 2023 discovered that over half of the respondents (64.2%) endorsed suicidality, and 22.4% had demonstrated suicidal behaviour. A 2024 research about high quality of life in power submit puncture sufferers (like me) discovered substantial psychological well being challenges with despair, nervousness and stress skilled by 83%, 98%, and 88% of the respondents, respectively.
It’s like an enormous cosmic joke, “hey, there’s a condition that often does not show on imaging, where specialized imaging also involves making a new hole in the dura mater to look for the original leak, where the vast majority of medical students don’t learn specifics of SIH or long-term puncture leaks in in current med school curricula, and where we can’t yet figure out why chronic patients have a really hard time getting sealed and sometimes end up with new leaks after treatment.”
***
August 2024 marks 7 years of leaking for me.
In these very tough intervening years, I’ve discovered lots of issues about myself, and about my physique. About the illusions of security I used to have. About how life simply isn’t truthful.
The half that hits hardest has at all times been that stability appears like an phantasm. It’s so tenuous. Moments the place I get respite from the ache are so few and much between, however with frequent anaphylaxis and extreme MCAS within the combine, it appears like the sting of that knife is so skinny. I usually joke that I’m in mattress now, however we are going to all be in mattress finally as we age. I simply bought there first. Underneath the humour is the information that Connie’s story is unfortunately not the exception. That there are different sufferers barely hanging on as they watch for science to evolve and be capable to convey them lasting aid.
Every day, my inbox is filled with sufferers who can’t get therapy for his or her spinal CSF leaks. Doctors who snicker at them for asking for atraumatic needles for a lumbar puncture, regardless of research exhibiting it makes a major distinction in reducing the danger of a post-puncture spinal CSF leak. Families who say that their youngsters or siblings or spouses are faking it, that they’re “just lazy”, that they only “don’t want to be better”.
Show me a chronically unwell particular person, in fixed ache, who doesn’t need to get higher?! If they’re on the market, they’re a rarity. Every affected person I do know, and I do know many on account of my public platforms, is like Connie: advocating fiercely, organizing effectively, maintaining hope as finest they will, and desperately desirous to get nicely.
I haven’t gone again for therapy as a result of repairs don’t maintain for me. I’ve complicating elements like MCAS and adhesive arachnoiditis that make outcomes extra dangerous and fewer healing than the common affected person. But that that doesn’t imply I’ve given up. I’ve stated it earlier than: acceptance doesn’t imply you’ve given up. Like Connie, I’ve a lot to be glad about. But even acceptance, even discovering pleasure once more, isn’t sufficient to eclipse unrelenting ache. Thankfully, I’m not within the form of ache Connie was in, and by sharing all of this I’m not attempting to make her dying about me. It’s extra that her passing feels prefer it may have been any of us on this tight knit neighborhood. I respect her selection, and I’m additionally heartbroken that she was backed right into a nook by her ache and felt like she needed to make it.
This submit isn’t my typical, I do know. I’m offended. I’m devastated. And it’s lit a fireplace beneath me to proceed my advocacy work as a lot as my physique permits. People typically ask why I’m so concerned with the Spinal CSF Leak Foundation within the US, because it’s unpaid and I’ve my very own enterprise to run. (I’m now the Vice-President of the Board). Advocacy is why. Connie is why. Connie, and different sufferers like her. Other sufferers like me. For these of us who’re power, the place repairs don’t maintain, who’re additionally ready and hoping. For the sufferers who can’t get to care as a result of they don’t have docs who know leaks of their areas. For those that want sources to assist their households to know.
In supporting the Foundation, and dealing with sister nonprofit organizations in Canada and the UK, I can hopefully result in extra change than I can do as one author with a leak.
I’ve been that means to share all of those ideas since I discovered of Connie’s passing earlier this month, nevertheless it actually messed me up an excessive amount of to place phrases to paper. Today marks the start of duradash®, although, and I’m fundraising for spinal CSF leak analysis. So I assumed it was an excellent day to share, and to try to mud myself off and preserve persisting. What else can I do however try to transmute my rage and my grief about Connie’s dying and the unfairness of all of it into one thing that may transfer the needle on all of this mess?
In August 2017, I knew nothing about spinal CSF, nothing about leaks aside from those in water pipes, and nothing about needle varieties or gauge. I had by no means heard of the dura mater. Years later, there’s lots I want I knew then, however all I can do is preserve writing and sharing, elevating funds for the trigger, and hopefully assist another person get care prior to I may.
I’ll at all times be grateful to Connie for her fierce willpower and want to teach others it doesn’t matter what she was going by way of.
Since I sustained my leak 7 years in the past, there was lots of progress and analysis and advances within the subject.
Losing Connie exhibits us how we have now a lot farther to go. 💔
If you’d wish to help me in elevating funds for spinal CSF leak analysis, please see my duradash® marketing campaign web page right here. I set a purpose of $1000 however have already eclipsed it due to my Instagram neighborhood; I might be upping the purpose to $2000 later this week. The 2-week fundraiser will finish June eighth, 2024.